Recurrent fevers
O1 Efficacy and safety of Canakinumab in patients with periodic fever syndromes (colchicine-resistant fmf, hids/mkd and traps): results from a phase 3, pivotal, umbrella trial
F. De Benedetti1, J. Anton2, M. Gattorno3, H. Lachmann4, I. Kone-Paut5, S. Ozen6, J. Frenkel7, A. Simon8, A. Zeft9, E. Ben-Chetrit10, H.M. Hoffman11, Y. Joubert12, K. Lheritier12, A. Speziale12, J. Guido12
1IRCCS Ospedale Pediatrico Bambino Gesú, Rome, Italy; 2Hospital Sant Joan de Déu, Barcelona, Spain; 3Pediatric Rheumatology, G. Gaslini Institute, Genoa, Italy; 4UK National Amyloidosis Centre, University College London Medical School, London, United Kingdom; 5Hôpital Kremlin Bicetre, University of Paris SUD, Paris, France; 6 Department of Pediatrics, Hacettepe University, Ankara, Turkey; 7University Medical Center Utrecht, Utrecht, Netherlands; 8Radboud University Medical Centre, Nijmegen, Netherlands; 9Pediatrics Rheumatology, Cleveland Clinic, Cleveland, USA; 10Rheumatology Unit, Hadassah—Hebrew University Medical Center, Jerusalem, Israel; 11University of California, San Diego, La Jolla, USA; 12Novartis Pharma AG, Basel, Switzerland
Presenting author: F. De Benedetti
Cohort | Canakinumab (150 mg q4w), n/m (%) | Placebo n/m (%) | p-value |
---|---|---|---|
crFMF | 19/31 (61.3) | 2/32 (6.3) | <0.001* |
HIDS/MKD | 13/37 (35.1) | 2/35 (5.7) | 0.0020* |
TRAPS | 10/22 (45.5) | 2/24 (8.3) | 0.0050* |
O2 An update on the Italian cohort of DADA2 patients and an assessment of a novel functional screening test
Roberta Caorsi1, Federica Penco1, Alice Grossi2, Antonella Insalaco3, Maria Alessio4, giovanni Conti5, Federico Marchetti6, Alberto Tommasini7, Silvana Martino8, Romina Gallizzi9, Annalisa Salis10, Francesca Schena1, Francesco Caroli2, Alberto Martini11, Gianluca Damonte10, Isabella Ceccherini2, Marco Gattorno1
1Second Division of Pediatrics, G. Gaslini Institute, Genova, Italy; 2Division of Human Genetics, G. Gaslini Institute, Genova, Italy; 3Division of Rheumathology, Bambino Gesù children hospital, Rome, Italy; 4Department of Pediatrics, Federico II Hospital, Napoli, Italy; 5Depatment of Pediatric rheumthology and nefrology, Policlinico di Messina, Messina, Italy, 6Department of Pediatrics, S. Maria delle Croci Hospital, Ravenna, Ravenna, Italy; 7Division of Rheumathology, Burlo Garofalo children hospital, Trieste, Italy; 8Department of Pediatrics, Regina Margherita Hospital, Torino, Italy; 9Department of Pediatrics, Policlinico di Messina, Messina, Italy; 10Department of cellular biochemistry, University of Genova, Genova, Italy; 11Scientific Director, G. Gaslini Institute, Genova, Italy
Presenting author: Roberta Caorsi
Mechanisms of disease
O3 Inhibition of interferon production driving tmem173-associated auto-inflammation
Marie-Louise Frémond1, Carolina Uggenti1, Lien Van Eyck1, Isabelle Melki1, Darragh Duffy2, Vincent Bondet2, Yoann Rose1, Bénédicte Neven3, Yanick Crow1, Mathieu P. Rodero1
1Laboratory of Neurogenetics and Neuroinflammation, Imagine Institute, Paris, France; 2Center for Human Immunology, Institut Pasteur, Paris, France; 3Pediatric Hematology-Immunology and Rheumatology Department, Hôpital Necker, Paris, France
Presenting author: Marie-Louise Frémond
O4 NLRP3 inflammasome activity in monocytes is regulated by 12/15-lipoxygenase
Yvonne Kusche1, Johannes Roth1, Katarzyna Barczyk-Kahlert1
1Institute of Immunology, Münster, Germany
Presenting author: Yvonne Kusche
Other autoinflammatory diseases
O5 PLAS: a novel autoinflammatory and lymphoproliferative syndrome associated with PIM-1 mutations
Giovanna Ferrara1, Annalisa Chiocchetti2, Silvio Polizzi3, Josef Vuch4, Diego Vozzi4, Anna Mondino4, Erica Valencic4, Serena Pastore4, Andrea Taddio4, Flavio Faletra4, Umberto Dianzani2, Ugo Ramenghi5, Alberto Tommasini4
1Department of Pediatrics, University of Trieste, Trieste, Italy; 2Amedeo Avogadro University of Eastern Piedmont, Novara, Italy; 3Ophthalmology Department, University of Florence, A.O.U.C., Florence, Italy; 4Department of Pediatrics, IRCCS Burlo Garofolo, Trieste, Italy; 5Department of Pediatric Sciences, University of Torino, Torino, Italy
Presenting author: Giovanna Ferrara
O6 Biallelic hypomorphic mutations in a linear deubiquitinase define otulipenia, an early-onset systemic autoinflammatory disease
Qing Zhou1, Xiaomin Yu2, Erkan Demirkaya3, Natalie Deuitch1, Deborah Stone1, Wanxia Tsai4, Amanda Ombrello1, Tina Romeo1, Elaine F. Remmers1, JaeJin Chae1, Massimo Gadina4, Steven Welch5, Seza Ozen6, Rezan Topaloglu6, Mario Abinun7, Daniel L. Kastner1, Ivona Aksentijevich1
1Inflammatory Disease Section, National Human Genome Research Institute, Bethesda, USA; 2Genetics and Pathogenesis of Allergy Section, National Institute of Allergy and Infectious Diseases, Bethesda, USA; 3FMF Arthritis Vasculitis and Orphan disease Research Center (FAVOR), Gulhane Military Medical Academy, Ankara, Turkey; 4Translational Immunology Section, National Institute of Arthritis and Musculoskeletal Diseases, Bethesda, USA; 5Heart of England NHS Foundation Trust, Birmingham, United Kingdom; 6Department of Pediatric Nephrology and Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey; 7Institute of Cellular Medicine, Newcastle University, Newcastle, United Kingdom
Presenting author: Qing Zhou
Diagnostic challenges
O7 Discrimination among monogenic and acquired disease with inflammatory and autoimmune component through the in vitro study of signal transducers of type I interferon
Donatella Vairo1, Rosalba Monica Ferraro1, Giulia Zani2, Jessica Galli3, Micaela De Simone3, Marco Cattalini4, Elisa Fazzi3, Silvia Giliani1
1Translational Molecular Medicine Department, University of Brescia, Brescia, Italy; 2Clinical and Expreimental Sciences, Pediatric Clinic, Brescia, Italy; 3Unit of Child Neurology and Psychiatry, Spedali Civili, Brescia, Italy; 4Clinical and Experimental Sciences, University of Brescia, Brescia, Italy
Presenting author: Donatella Vairo
O8 A targeted next-generation sequencing gene panel for autoinflammation
Ebun Omoyinmi1, Ariane Standing1, Dorota Rowczenio2, Annette Keylock1, Sonia Melo Gomes1, Fiona Price-Kuehne1, Sira Nanthapisal1, Claire Murphy1, Thomas Cullup3, Lucy Jenkins3, Kimberly Gilmour4, Despina Eleftheriou1, Helen Lachmann2, Philip Hawkins2, Nigel Klein1, Paul Brogan1
1IIIP, UCL, Institute of Child Health, London, United Kingdom; 2NAC, Royal Free Hospital, London, United Kingdom; 3NE Thames Genetics Laboratory, London, United Kingdom; 4Immunology, GOSH, London, United Kingdom
Presenting author: Ebun Omoyinmi
Vasculitis
O9 Optimal aspirin dose in Kawasaki disease - the ongoing battle
Anita Dhanrajani1, Mercedes Chan2, Stephanie Pau3, Janet Ellsworth2, Jaime Guzman1
1Pediatric Rheumatology, BC Children’s Hospital, University of British Columbia, Vancouver, Canada; 2Pediatric Rheumatology, Stollery Children’s Hospital, University of Alberta, Edmonton, Canada; 3Pediatrics, Stollery Children’s Hospital, Edmonton, Canada
Presenting author: Anita Dhanrajani
Unadjusted | Adjusted | |||||||
---|---|---|---|---|---|---|---|---|
Variable names | ODDS RATIO | 95% CI lower | 95% CI upper | P -value | ODDS RATIO | 95% CI lower | 95% CI upper | P- value |
Aspirin dose (HIGH vs LOW) | 0.318 | 0.151 | 0.673 | 0.003 | 0.313 | 0.110 | 0.892 | 0.030 |
Platelet count <300000 IN WEEK 1 | 1.585 | 0.889 | 2.827 | 0.119 | 1.734 | 0.889 | 3.384 | 0.107 |
CRP value | 1.852 | 0.305 | 11.258 | 0.503 | 1.003 | 0.998 | 1.008 | 0.267 |
Age categories (<=12 MONTHS, >12 MONTHS) | 1.138 | 0.444 | 2.916 | 0.788 | 1.096 | 0.379 | 3.173 | 0.865 |
Sodium < 133 mmol/L | 1.368 | 0.711 | 2.631 | 0.348 | 1.752 | 0.842 | 3.646 | 0.134 |
O10 Childhood Takayasu Arteritis: disease course and response to therapy
Florence A. Aeschlimann1, Marinka Twilt2, Simon W. Eng1, Shehla Sheikh1, Ronald M. Laxer1, Diane Hebert3, Damien Noone3, Christian Pagnoux4, Susanne M. Benseler2, Rae S. Yeung1
1Rheumatology, The Hospital for Sick Children, Toronto, Canada; 2Rheumatology, Alberta Children’s Hospital, Calgary, Canada; 3Nephrology, The Hospital for Sick Children, Toronto, Canada; 4Rheumatology, Mount Sinai Hospital, Toronto, Canada
Presenting author: Susanne M. Benseler
Induction therapy | No. of patients (Total N = 27) | Median PVAS at diagnosis (range) | Inactive disease at 6 months | Therapy for maintenance of remission/ during disease course | No. of patients with flares | Death | Inactive disease at last follow up |
---|---|---|---|---|---|---|---|
CS only | 4 (15) | 16 (8–17) | 1/3 | CS, Cyclo, Aza, MMF, MTX | 3/4 | 1/4 | 1/3 |
CS + MTX | 10 (37) | 15.5 (6–21) | 2/10 | CS, Cyclo, Aza, Lef, MTX, Biologics | 7/10 | 0/10 | 5/10 |
CS + Cyclo | 5 (19) | 7 (6–35) | 2/4 | CS, Cyclo, Aza, Lef, MTX, Biologic | 3/5 | 1/5 | 3/4 |
CS + MTX + Biologic | 3 (11) | 10 (6–25) | 3/3 | CS, Biologic + Lef/MTX | 0/3 | 0/3 | 3/3 |
No treatment | 5 (19) | 10 (6–17) | 5/5 | None | 0/5 | 0/5 | 5/5 |
Immune aspects of JIA
O11 Dysbalanced serum factor environments can drive IL-17 over-expression by gamma delta T cells in systemic juvenile idiopathic arthritis
Christoph Kessel1, Katrin Lippitz1, Toni Weinhage1, Claas Hinze1, Helmut Wittkowski1, Dirk Holzinger1, Niklas Grün1, Dirk Föll1
1Pediatric Rheumatology & Immunology, University Childrens’ Hospital, Münster, Germany
Presenting author: Christoph Kessel
O12 The composition of the gut microbiota differs between children with JIA and healthy controls
Pieter Van Dijkhuizen1,2, Federica Del Chierico3, Clara Malattia1,4, Alessandra Russo3, Denise Pires Marafon5, Nienke M. ter Haar2, Silvia Magni-Manzoni5, Sebastiaan J. Vastert2, Bruno Dallapiccola, Berent Prakken2, Alberto Martini1,4, Fabrizio De Benedetti5, Lorenza Putignani3
1Paediatric rheumatology, Istituto Giannina Gaslini, Genoa, Italy; 2Paediatric rheumatology, University Medical Centre Utrecht, Wilhelmina Children’s Hospital, Utrecht, Netherlands; 3Human Microbiome Unit, Ospedale Pediatrico Bambino Gesù, Rome, Italy; 4Università degli Studi di Genova, Genoa, Italy; 5Paediatric rheumatology, Ospedale Pediatrico Bambino Gesù, Rome, Italy; 6Scientific direction, Ospedale Pediatrico Bambino Gesù, Rome, Italy
Presenting author: Pieter Van Dijkhuizen
Vasculitis and Behcet disease
O13 A new approach to assessment of juvenile vasculitis: the juvenile vasculitis multidimensional assessment report (J-VAMAR)
Berna Eren Fidanci1, Kenan Barut2, Serap Arıcı3, Dogan Simsek1, Mustafa Cakan4, Ezgi D. Batu3, Sezgin Şahin2, Ayşenur Kısaarslan5, Ebru Yilmaz6, Özge Basaran7, Ferhat Demir8, Kubra Ozturk9, Zübeyde Gunduz5, Betül Sozeri5, Balahan Makay10, Nuray Ayaz4, Onder Yavascan6, Ozlem Aydog11, Yelda Bilginer3, Zelal Ekinci9, Dilek Yıldız1, Faysal Gök1, Muferret Erguven12, Erbil Unsal10, Ozgur Kasapcopur2, Seza Ozen3, Erkan Demirkaya1, and For the FMF Arthritis Vasculitis and Orphan Disease Research in Paediatric Rheumatology (FAVOR)
1Gulhane Military Medical Academy, Ankara, Turkey; 2Istanbul University, Istanbul, Turkey; 3Hacettepe University, Ankara, Turkey; 4Kanuni Sultan Suleyman Research Hospital, Istanbul, Turkey; 5Erciyes University, Kayseri, Turkey; 6Tepecik Training Hospital, Izmir, Turkey; 7Dıskapı Training Hospital, Ankara, Turkey; 8Karadeniz Technical University, Trabzon, Turkey; 9Kocaeli University, Kocaeli, Turkey; 109 Eylul University, Izmir, Turkey; 11Sami Ulus Children Hospital, Ankara, Tukey; 12Goztepe Training Hospital, Istanbul, Turkey
Presenting author: Berna Eren Fidanci
O14 Behçet’s disease in children: Eastern Mediterranean experience
Hafize E. Sönmez1, Ezgi D. Batu1, Betül Sözeri2, Yonatan Butbul3, Yelda Bilginer1, Seza Özen1
1Division of Rheumatology, Hacettepe University Faculty Of Medicine, Ankara, Turkey; 2Division of Rheumatology, Erciyes University Faculty of Medicine, Kayseri, Turkey; 3Division of Rheumatology, Rambam Medical Center, Haifa, Israel
Presenting author: Hafize E. Sönmez
Macrophage activation syndrome
O15 Immune abnormalities leading to exaggerated production of IFN-gamma and the therapeutic response to an anti-IFN-gamma antibody in a patient with NLRC4 mediated disease
Claudia Bracaglia1#, Giusi Prencipe1#, Manuela Pardeo1, Geneviève Lapeyre2, Emiliano Marasco1, Antonella Insalaco1, Walter Ferlin2, Robert Nelson2, Cristina de Min2, Fabrizio De Benedetti1
1Division of Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy; 2Novimmune SA, Geneva, Switzerland
Presenting author: Claudia Bracaglia
O16 Long-term efficacy and safety of Canakinumab in patients with active systemic juvenile idiopathic arthritis (SJIA): results from a phase III extension study
N. Ruperto1, H.I. Brunner2, P. Quartier3, T. Constantin1, E. Alexeeva1, I. Kone-Paut1, K. Marzan2, N. Wulffraat1, R. Schneider2, S. Padeh1, V. Chasnyk1, C. Wouters1, J.B. Kuemmerle-Deschner1, T. Kallinich1, B. Lauwerys4, E. Haddad2, E. Nasonov1, M. Trachana1, O. Vougiouka1, K. Leon5, E. Vritzali6, K. Lheritier6, A. Martini1, D. Lovell2, and PRINTO/PRCSG
1PRINTO-Istituto Gaslini, Genoa, Italy; 2PRCSG, Cincinnati, OH, USA; 3Necker-Enfants Malades Hospital, Paris, France; 4Cliniques Universitaires Saint-Luc and Université Catholique de Louvain, Brussels, Belgium; 5Novartis Pharmaceuticals Corporation, East Hanover, NJ, USA; 6Novartis Pharma AG, Basel, Switzerland
Presenting author: N. Ruperto
Time point | CAN | ||
---|---|---|---|
N = 123 | |||
Minimum adapted ACR pediatric response | n (n/m%) | Patients with inactive disease (n/m)% | |
Month 12 | m | 85 | (52/88) 59.1 |
Non-Responders | 4 (4.7) | ||
aACR ≥30 | 81 (95.3) | ||
aACR ≥50 | 77 (90.6) | ||
aACR ≥70 | 73 (85.9) | ||
aACR 100 | 49 (57.6) | ||
Month 21 | m | 65 | (48/65) 73.8 |
Non-Responders | 3 (4.6) | ||
aACR ≥30 | 62 (95.4) | ||
aACR ≥50 | 58 (89.2) | ||
aACR ≥70 | 54 (83.1) | ||
aACR 100 | 39 (60.0) | ||
Last assessment | m | 121 | (62/122) 50.8 |
Non-Responders | 28 (23.1) | ||
aACR ≥30 | 93 (76.9) | ||
aACR ≥50 | 89 (73.6) | ||
aACR ≥70 | 81 (66.9) | ||
aACR 100 | 62 (51.2) |
Oral presentations 1
O17 Defect of adaptive immunity in ADA2 deficiency patients
Francesca Schena1, Stefano Volpi1, Roberta Caorsi1, Federica Penco1, Claudia Pastorino1, Francesca Kalli2, Alessia Omenetti1, Sabrina Chiesa1, Arinna Bertoni1, Paolo Picco1, Gilberto Filaci2, Ivona Aksentijevich3, Alice Grossi4, Isabella Ceccherini4, Alberto Martini1, Elisabetta Traggiai5, Marco Gattorno1
1Second Pediatric Division, Giannina Gaslini Institute, Genova, Italy; 2CEBR, Università, Genova, Italy; 3National Human Genome Research Institute, National Institute of Health, Bethesda, MD, USA; 4Medical Genetics, G. Gaslini Institute, Genova, Italy; 5Novartis Institute for Research in Biomedicine, Basel, Switzerland
Presenting author: Francesca Schena
O18 Previously undescribed SAVI-associated mutations identify a novel region in human sting necessary for the control of type I interferon signalling
Isabelle Melki1,2,3, Yoann Rose1, Carolina Uggenti1, Marie-Louise Fremond1, Lien Van Eyck1, Naoki Kitabayashi1, Marco Gattorno4, Stefano Volpi4, Olivero Sacco5, Isabelle Meyts6, Marie-Anne Morren7, Carine Wouters8, Eric Legius9, Isabelle Callebaut10, Christine Bodemer11, Frederic Rieux-Laucat12, Mathieu Rodero1, Yanick Crow1
1Laboratory of Neurogenetics and Neuroinflammation, Imagine Institute, Paris, France; 2General Pediatrics - Internal Medicine - Infectious diseases, Robert Debré Hospital - APHP, Paris, France; 3Pediatric Immuno-Haematology and Rheumatology Unit, Necker Hospital - APHP, Paris, France; 4UO Pediatria 2 Reumatologia, Genova, Italy; 5UO Pneumologia, G. Gaslini, Genova, Italy; 6Immunology, UZLeuven, Leuven, Belgium; 7Dermatology, UZLeuven, Leuven, Belgium; 8Rheumatology, UZLeuven, Leuven, Belgium; 9Genetics, UZLeuven, Leuven, Belgium; 10MPMC, Sorbonne University — UMR CNRS 7590, UPMC Sorbonne Paris Cité, Muséum National d’Histoire Naturelle, Paris, France, IRD UMR 206, Paris, France; 11Dermatology, Necker Hospital - APHP, Paris, France; 12INSERM UMR 1163; Laboratory of Immunogenetics of Pediatric Autoimmunity, Imagine Institute, Paris, France
Presenting author: Isabelle Melki
O19 Efficacy of the Janus kinase 1/2 inhibitor ruxolitinib in the treatment of vasculopathy associated with TMEM173-activating mutations in three children
Marie-Louise Frémond1, Mathieu P. Rodero1, Nadia Jeremiah2, Alexandre Belot3, Eric Jeziorski4, Darragh Duffy5, Didier Bessis6, Guilhem Cros7, Gillian I. Rice8, Bruno Charbit5, Anne Hulin9, Nihel Khoudour9, Consuelo Modesto Caballero10, Christine Bodemer11, Monique Fabre12, Laureline Berteloot13, Muriel Le Bourgeois14, Philippe Reix15, Thierry Walzer16, Despina Moshous7, Stéphane Blanche7, Alain Fischer7, Brigitte Bader-Meunier7, Frédéric Rieux-Laucat2, Yanick Crow1, Bénédicte Neven7
1Laboratory of Neurogenetics and Neuroinflammation, Paris, France; 2Laboratory of Immunogenetics of Pediatric Autoimmunity, Imagine Institute, Paris, France; 3Pediatric Rheumatology, Nephrology and Dermatology Department, Hospices Civils de Lyon, Lyon, France; 4Pediatrics Department, Centre Hospitalier Universitaire de Montpellier, Montpellier, France; 5Laboratory of Dendritic Cell Immunobiology, Institut Pasteur, Paris, France; 6Dermatology Department, Centre Hospitalier Universitaire de Montpellier, Montpellier, France; 7Pediatric Hematology-Immunology and Rheumatology Department, Hôpital Necker-Enfants Malades, Paris, France; 8Manchester Centre for Genomic Medicine, University of Manchester, Manchester, United Kingdom; 9Pharmacology and Toxicology Laboratory, Hôpital Universitaire Henri Mondor, Créteil, France; 10Pediatric Rheumatology Department, Hospital Universitari Vall d’Hebron, Barcelona, Spain; 11Dermatology Department, Hôpital Necker-Enfants Malades, Paris, France; 12Pathology Department, Hôpital Necker-Enfants Malades, Paris, France; 13Pediatric Radiology Department, Hôpital Necker-Enfants Malades, Paris, France; 14Pediatric Pneumology Department, Hôpital Necker-Enfants Malades, Paris, France; 15Pneumology Department, Hospices Civils de Lyon, Lyon, France; 16Centre International de Recherche en Infectiologie, Université Lyon 1, Lyon, France
Presenting author: Marie-Louise Frémond
O20 Development of the autoinflammatory disease damage index (ADDI)
K Annink1, N ter Haar1, S Al-Mayouf2, G Amaryan2, J Anton2, K Barron2, S Benseler2, P Brogan2, L Cantarini2, M Cattalini2, A Cochino2, F De Benedetti2, F Dedeoglu2, A De Jesus2, O Dellacasa2, E Demirkaya2, P Dolezalova2, K Durrant3, G Fabio2, R Gallizzi2, R Goldbach-Mansky2, E Hachulla2, V Hentgen2, T Herlin2, M Hofer2, H Hoffman2, A Insalaco2, A Jansson2, T Kallinich2, I Koné-Paut2, A Kozlova2, J Kuemmerle-Deschner2, H Lachmann2, R Laxer2, A. Martini2, S Nielsen2, I Nikishina2, A Ombrello2, S Ozen2, E Papadopoulou-Alataki2, P Quartier2, A Ravelli2, D Rigante2, R Russo2, A Simon2, M Trachana2, Y Uziel2, M Gattorno2, J Frenkel1
1UMC Utrecht, Utrecht, Netherlands; 2The ADDI consortium, Genua, Italy; 3Autoinflammatory Alliance, San Fransisco, CA, USA
Presenting author: N ter Haar
O21 The phenotype and genotype of mevalonate kinase deficiency: a series of 114 cases from the Eurofever registry
Nienke ter Haar1, Jerold Jeyaratnam1, Helen Lachmann1, Anna Simon1, Paul Brogan1, Matteo Doglio1, Marco Cattalini1, Jordi Anton1, Consuelo Modesto1, Pierre Quartier1, Esther Hoppenreijs1, Silvana Martino1, Antonella Insalaco1, Luca Cantarini1, Loredana Lepore1, Maria Alessio1, Inmaculada Calvo Penades1, Christina Boros1, Rita Consolini1, Donato Rigante1, Ricardo Russo1, Jana Pachlopnik Schmid1, Thirusha Lane1, Alberto Martini1, Nicolino Ruperto1, Joost Frenkel1, Marco Gattorno1
1Eurofever Project, University Medical Center Utrecht, Utrecht, Netherlands
Presenting author: Jerold Jeyaratnam
O22 Autoinflammatory diseases: role of next-generation sequencing
Chiara Passarelli1, Elisa Pisaneschi1, Virginia Messia2, Manuela Pardeo2, Antonio Novelli1, Fabrizio Debenedetti2, Antonella Insalaco2
1Division of Medical Genetics, Roma, Italy; 2Division of Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy
Presenting author: Chiara Passarelli
O23 Effectiveness of childhood vaccinations in CAPS patients treated with Canakinumab: results from an open-label phase 3 extension study
P.A. Brogan1, M. Hofer2, J.B. Kuemmerle-Deschner3, B. Lauwerys4, A. Speziale5, X. Wei6, R. Laxer7
1UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, Department of Paediatric Rheumatology, London, United Kingdom; 2Unité Romande de Rhumatologie Pédiatrique, Hospitalier Universitaire Vaudois, Lausanne, Switzerland; 3University Hospital Tuebingen, Tuebingen, Germany; 4Cliniques Universitaires Saint-Luc and Université Catholique de Louvain, Brussels, Belgium; 5Novartis Pharma AG, Basel, Switzerland; 6Novartis Pharma, Beijing, China; 7University of Toronto, The Hospital for Sick Children, Ontario, Canada
Presenting author: P.A. Brogan
O24 Baseline characteristics of 116 patients with cronic non-bacterial osteomyelitis (CNO) included in a national longitudinal cohort study
Antonella Insalaco1, Denise Pires Marafon1, Martina Finetti2, Manuela Pardeo3, Silvana Martino4, Marco Cattalini5, Maria Alessio6, Francesca Orlando7, Andrea Taddio7, Serena Pastore7, Elisabetta Cortis8, Angela Miniaci9, Nicola Ruperto2, Alberto Martini2, Fabrizio De Benedetti1, Marco Gattorno2
1Division of Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy; 2Pediatria II Reumatologia PRINTO, Istituto Giannina Gaslini, Genova, Italy; 3Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, Roma, Italy; 4Paediatrics Departments, Clinica Pediatrica Universita’ di Torino, Torino, Italy; 5Unità di immunologia e Reumatologia Pediatrica, Clinica Pediatrica Università Brescia, Spedali Civili, Brescia, Italy; 6Dipartimento di Pediatria, Università di Napoli Federico II, Napoli, Italy; 7Scienze della Riproduzione e dello Sviluppo, Università di Trieste IRCCS Burlo Garofalo, Trieste, Italy; 8Struttura Complessa Pediatria, Ospedale Santa Maria della Stella, Orvieto, Italy; 9Ambulatorio di Reumatologia, Aziensa Ospedaliera Universitaria Sant’Orsola Malpighi, Bologna, Italy
Presenting author: Antonella Insalaco
O25 Analyses of 247 patients with recurrent inflammation of unknown cause
Charlotte Eijkelboom1, Nienke ter Haar1, Luca Cantarini2, Martina Finetti3, Paul Brogan4, Pavla Dolezalova5, Isabelle Koné-Paut6, Antonella Insalaco7, Marija Jelusic-Drazic8, Liliana Bezrodnik9, Mari Carmen Pinedo10, Valda Stanevicha11, Marielle van Gijn1, Silvia Federici3, Nicolino Ruperto3, Joost Frenkel1, Marco Gattorno3
1UMCU, Utrecht, Netherlands; 2University of Siena, Siena, Italy; 3IGG, Genova, Italy; 4GOSH, London, United Kingdom; 5General University Hospital, Prague, Czech Republic; 6Bicêtre hospital, Le Kremlin-Bicêtre, France; 7OPBG, Rome, Italy; 8University Hospital Centre, Zagreb, Croatia; 9Hospital de Ninos Ricardo Gutierrez, Buenos Aires, Argentina; 10Hospital de Cruces, Barakaldo, Spain; 11Stradins University, Riga, Latvia
Presenting author: Charlotte Eijkelboom
Alwaysa
| Sometimes/oftena
| Most common complaintsa
| |
---|---|---|---|
Muco-cutaneous | 22 | 47 | Aphthous stomatis (27) |
Gastro-intestinal | 14 | 41 | Abdominal pain (47) |
Musculoskeletal | 21 | 46 | Arthralgia (61), myalgia (44) |
Ocular | 2 | 12 | Conjunctivitis (8) |
Lymphoid | 33 | 14 | Enlarged cervical lnn (38) |
Cardio-respiratory | 3 | 15 | Chest pain (13) |
Neurological | 15 | 26 | Headache (32) |
Genito-urinary | 1 | 5 | Urethritis/cystitis (3) |
Constitutional | 25 | 40 | Fatigue (55), malaise (49) |
O26 The multifaceted presentation of chronic recurrent multifocal osteomyelitis: a series of 466 cases from the Eurofever international registry
Hermann Girschick1, Martina Finetti2, Francesca Orlando2,3, Antonella Insalaco4, Gerd Ganser5, Susan Nielsen6, Troels Herlin7, Isabelle Koné-Paut8, Silvana Martino9, Marco Cattalini10, Jordi Anton11, Sulaiman Mohammed Al-Mayouf12, Michael Hofer13, Pierre Quartier14, Christina Boros15, Jasmin Kuemmerle-Deschner16, Susanne Schalm17, Maria Alessio18, Nicolino Ruperto2, Alberto Martini19, Annette Jansson20, Marco Gattorno2, on behalf of PRINTO and Eurofever registry
1 Perinatal Centre of the Vivantes Klinikum, Clinic for Paediatric and Adolescent Medicine, Berlin, Germany; 2Pediatria II, Reumatologia; PRINTO, Istituto Giannina Gaslini, Genoa, Italy; 3Università di Napoli Federico II, Naples, Italy; 4Ospedale Pediatrico Bambino Gesù, Rome, Italy; 5St. Josef-Stift Hospital, Sendenhorst, Germany; 6Rigshospitalet, Copenhagen, Denmark; 7Skejby Sygehus, Aarhus University Hospital, Aarhus, Denmark; 8Le Kremlin-Bicêtre University Hospital, Paris-Sud University, Paris, France; 9Dipartimento di Scienze Pediatriche e dell’Adolescenza, University of Torino, Torino, Italy; 10Unita’ di Immunologia e Reumatologia Pediatrica, Clinica Pediatrica dell’Universita’ di Brescia, Spedali Civili, Brescia, Italy; 11Hospital Sant Joan de Déu, Barcelona, Spain; 12King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia; 13Centre Multisite Romand de Rhumatologie Pediatrique / Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland; 14Paris-Descartes University, IMAGINE Institute, Necker Children’s Hospital, Paris, France; 15Women’s and Children’s Hospital, Adelaide, Australia; 16University Children’s Hospital Tuebingen, Tuebingen, Germany; 17Klinikum der Univeristat, Munchen, Germany; 18Dipartimento di Pediatria, Università di Napoli Federico II, Naples, Italy; 19Pediatria II, Reumatologia; PRINTO, Istituto Giannina Gaslini, Genoa, Italy; 20University Medical Centre, Munich, Germany
Presenting author: Hermann Girschick
O27 Clinical features distinguishing patients with Chronic Recurrent Multifocal Osteomyelitis (CRMO) from patients with tumoral osteolytic lesions: a single center experience
Martina Finetti1, Marta Marchi2, Chiara Marini2, Matteo Doglio2, Clara Malattia1, Angelo Ravelli1, Alberto Martini1, Alberto Garaventa3, Marco Gattorno1
1Pediatria II, Reumatologia, Istituto Giannina Gaslini, Genoa, Italy; 2Università di Genova, Genoa, Italy; 3Pediatria IV, Oncologia, Istituto Giannina Gaslini, Genoa, Italy
Variables | CRMO | LCH | P | Odds ratio (OR) | |
---|---|---|---|---|---|
Demographic manifestations | Mean onset age (years) | 9,6 | 4,3 | <0,001 | |
Clinical Manifestations | Musculoskeletal pain | 48/48 (100%) | 10/21 (48%) | <0,0001 | 0,06 (0,01/0,26) |
Swelling and/or functional limitations | 31/48 (65%) | 7/21 (33%) | <0,02 | 0,27 (0,09/0,81) | |
MR lesions site | Clavicle | 11/48 (22,9%) | 0/21 (0%) | <0,01 | |
Distal Fibula | 9/48 (19%) | 0/21 (0%) | <0,05 | ||
Foot | 10/48 (20,8%) | 0/21 (0%) | <0,03 | ||
Mean number of MR lesions | 6,5 | 2 | <0,03 |
O28 Cryopyrin associated periodic syndromes (CAPS): immunological characterization of knock-in mouse model to exploit novel approaches for the modulation of the NLRP3 inflammasome
Arinna Bertoni1, Sonia Carta2, Enrica Balza 2, Patrizia Castellani2, Caterina Pellecchia2, Federica Penco1, Francesca Schena1, Silvia Borghini3, Maria Libera Trotta4, Claudia Pastorino1, Isabella Ceccherini3, Alberto Martini5, Marco Gattorno5, Anna Rubartelli2, Sabrina Chiesa1
1Laboratorio di Immunologia delle Malattie Reumatiche, Giannina Gaslini Institute, Genova, Italy; 2Unità di Biologia Cellulare, IRCCS San Martino-Ist, Genova, Italy; 3Genetica Medica, Giannina Gaslini Institute, Genova, Italy; 4Università degli Studi di Genova, Genova, Italy; 52° Pediatric Division, Giannina Gaslini Institute, Genova, Italy
Presenting author: Arinna Bertoni
Oral presentations 2
O29 Predicting which children with juvenile idiopathic arthritis will have a severe disease course: results from the ReACCh-Out cohort
Jaime Guzman1, Andrew Henrey2, Thomas Loughin2, Roberta Berard3, Natalie Shiff4, Roman Jurencak5, Susanne Benseler6, Lori Tucker1, on behalf of ReACCh-Out Investigators
1Pediatrics, University of British Columbia, Vancouver, Canada; 2Statistics, Simon Fraser University, Burnaby, Canada; 3Pediatrics, Western University, London, Canada; 4Pediatrics, University of Florida, Gainesville, FL, USA; 5Pediatrics, University of Ottawa, Ottawa, Canada; 6Pediatrics, University of Calgary, Calgary, Canada
Presenting author: Jaime Guzman
Decile of risk | 1) Logistic | 2) Random forest | 3) K-nearest neighbor | 4) Neural network | 5) JIA category alone |
---|---|---|---|---|---|
First (low) | 0.05 | 0.04 | 0.16 | 0.11 | 0.05 |
Third | 0.05 | 0.05 | 0.05 | 0.09 | 0.06 |
Sixth | 0.10 | 0.13 | 0.17 | 0.10 | 0.32 |
Ninth | 0.43 | 0.45 | 0.29 | 0.41 | 0.36 |
Tenth (high) | 0.91 | 0.88 | 0.51 | 0.74 | 0.37 |
C-Index (95% CI) | 0.85 (0.80, 0.90) | 0.85 (0.82, 0.88) | 0.67 (0.59, 0.75) | 0.75 (0.71, 0.79) | 0.71 (0.65, 0.79) |
Maximum likelihood (95% CI) | −51 (−43, −59) | −58 (−49, −66) | −81 (−61, −100) | −107 (−99, −116) | −69 (−60, −77) |
Pearson (95% CI) | 47 (34, 61) | 45 (37, 53) | 12 (3, 20) | 26 (18, 33) | 13 (7, 19) |
O30 The vasculopathy of JDM; evidence of persistent endothelial injury, hypercoagulability, subclinical inflammation and increased arterial stiffness
Charalampia Papadopoulou1, Ying Hong1, Petra Krol1, Yiannis Ioannou2, Clarissa Pilkington1, Hema Chaplin2, Stephania Simou1, Marietta Charakida3, Lucy Wedderburn1,2, Paul Brogan1, Despina Eleftheriou1,2
1Department of Paediatric Rheumatology, Great Ormond Street Hospital and UCL Institute of Child Health, London, United Kingdom; 2Arthritis Research UK, Centre for Adolescent Rheumatology, London, United Kingdom; 3Institute of Cardiovascular Sciences, University College London, London, United Kingdom
Presenting author: Charalampia Papadopoulou
O31 Online peer mentoring for adolescents with juvenile idiopathic arthritis: the iPeer2Peer Program
Lynn R. Spiegel1,2, Sara Ahola Kohut3,4, Jennifer Stinson5,6, Paula Forgeron7, Miriam Kaufman2,8, Nadia Luca9, Khush Amaria3, Mary Bell10
1Rheumatology, The Hospital for Sick Children, Toronto, Canada; 2Pediatrics, University of Toronto, Toronto, Canada; 3Psychology, University of Toronto, Toronto, Canada; 4Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada; 5Child and Evaluative Health Sciences, The Hospital for Sick Children, Toronto, Canada; 6Lawrence S. Bloomberg Faculty of Nursing, University of Toronto, Toronto, Canada; 7School of Nursing, University of Ottawa, Ottawa, Canada; 8Adolescent Medicine, The Hospital for Sick Children, Toronto, Canada; 9Rheumatology, Alberta Children's Hospital, Calgary, Canada; 10Rheumatology, Sunnybrook Research Institute, Toronto, Canada
Presenting author: Lynn R. Spiegel
O32 Adjudication of infections in the pharmacovigilance in juvenile idiopathic arthritis patients (Pharmachild) treated with biologic agents and/or methotrexate
J Swart, F Boris, E Castagnola, A Groll, G Giancane, G Horneff, H-I. Huppertz, D Lovell, T Wolfs, M Hofer, E Alekseeva, V Panaviene, S Nielsen, J Anton, F Uettwiller, V Stanevicha, M Trachana, F De Benedetti, L M Ailioaie, E Tsitami, S Kamphuis, T Herlin, P Dolezalova, G Susic, F Sztajnbok, B Flato, A Pistorio, A Martini, N Wulffraat, N Ruperto
PRINTO, Genova, Italy
Presenting author: J Swart
O33 The influence of early achievement of clinically inactive disease on long-term disability outcomes in juvenile idiopathic arthritis
Stephanie J. W. Shoop1,2, Suzanne M. M. Verstappen1, Janet E. McDonagh3, Wendy Thomson2,4, Kimme L. Hyrich1,2, and CAPS
1Arthritis Research UK Centre for Epidemiology, The University of Manchester, Manchester, United Kingdom; 2NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester University Hospitals NHS Foundation Trust and University of Manchester Partnership, Manchester, United Kingdom; 3Centre for MSK Research, Faculty of Medical and Human Sciences, Manchester, United Kingdom; 4Arthritis Research UK Centre for Genetics and Genomics, The University of Manchester, Manchester, United Kingdom
Presenting author: Stephanie J. W. Shoop
CID state at one year following diagnosis | Mean CHAQ at five years following diagnosis (95% CI) | Percent with CHAQ ≤ 0.5 at five years following diagnosis (95% CI) |
---|---|---|
CID on Wallace’s preliminary criteria and cJADAS | 0.7 (0.5, 0.8) | 51 (40, 63) |
CID only on Wallace’s preliminary criteria | 1.0 (0.8, 1.3) | 33 (16, 50) |
No CID | 0.9 (0.8, 1.0) | 37 (29, 44) |
O34 Cost-effectiveness of first year treatment for juvenile idiopathic arthritis (JIA) in the era of biosimilars
Maarit Tarkiainen1,2, Pirjo Tynjala3, Pekka Lahdenne4, Janne Martikainen5, and Acute-JIA Study Group
1Institute of Clinical Medicine, University of Helsinki, Helsinki, Finland; 2Children’s Hospital, Helsinki, Finland; 3Poison Centre, Helsinki, Finland; 4Helsinki University Central Hospital, Helsinki, Finland; 5University of Eastern Finland, Kuopio, Finland
Presenting author: Maarit Tarkiainen
Treatment | Incremental Costs, € | Incremental QALYs | ICER estimate €/Qaly |
---|---|---|---|
COMBO vs MTX | -2918 | 0.072 | dominanta
|
aTNF vs combo | 7627 | 0.023 | 331 609 |
aTNF vs MTX | 4926 | 0.095 | 51 853 |
O35 Peripheral blood B cells are expanded and their cytokine expression is dysregulated in juvenile dermatomyositis
Meredyth Wilkinson1, Christopher Piper2, Georg Otto3, Claire T. Deakin3, Stefanie Dowle3, Stefania Simou3, Daniel Kelberman3, Yiannis Ioannou1, Claudia Mauri2, Elizabeth Jury2, David Isenberg2, Lucy R. Wedderburn3, Kiran Nistala3
1Centre for Adolescent Rheumatology, UCL, London, United Kingdom; 2Centre of Rheumatology, UCL, London, United Kingdom; 3UCL Institute of Child Health, UCL, London, United Kingdom
Presenting author: Meredyth Wilkinson
O36 Uveitis associated to polyarticular juvenile idiopathic arthritis (PJIA): data from strive registry
I Foeldvari1, N Ruperto1, DJ Lovell2, G Horneff1, H-I Huppertz1, P Quartier3, G Simonini1, M Bereswill4, J Kalabic4, A Martini1, HI Brunner2
1PRINTO-IRCCS, Genova, Italy; 2PRCSG, Cincinnati, OH, USA; 3Hôpital Necker-Enfants Malades, Paris, France; 4AbbVie Deutschland GmbH & Co. KG, Ludwigshafen, Germany
Presenting author: I Foeldvari
O37 Changes in quality of life over time in children with juvenile idiopathic arthritis: results from the ReACCh-Out cohort
Kiem Oen1, Jaime Guzman2, Brian M. Feldman3, Brenden Dufault4, Jennifer Lee5, Natalie Shiff6, Karen Watanabe Duffy5, Lori Tucker2, Ciaran Duffy5, and ReACCh-Out Investigators
1Pediatrics, University of Manitoba, Winnipeg, Canada; 2Pediatrics, University of British Columbia, Vancouver, Canada; 3Pediatrics, University of Toronto, Toronto, Canada; 4Statistics, University of Manitoba, Winnipeg, Canada; 5Pediatrics, University of Ottawa, Ottawa, Canada; 6Pediatrics, University of Florida, Gainesville, USA
Presenting author: Jaime Guzman
Total JAQQ | Gross motor | Fine motor | Psycho-social | Systemic Symptoms | QoML | Active jointsa
| Physician globalb
| CHAQc
| |
---|---|---|---|---|---|---|---|---|---|
Median score first visit (IQR) | 2.7 (2, 4) | 3.2 (2, 5) | 1.4 (1, 3) | 2.4 (1, 3) | 3.6 (2, 5) | 7.8 (5, 9) | 2 (1, 6) | 2.8 (1, 5) | 0.4 (0.1, 1) |
Median months to best score | 59.3 | 21.2 | 7.2 | 21.8 | 40.1 | 34.5 | 11.4 | 14.9 | 14 |
Probability of best score within 1 year | 0.10 | 0.32 | 0.61 | 0.33 | 0.15 | 0.25 | 0.53 | 0.38 | 0.44 |
Probability of best score within 2 years | 0.24 | 0.52 | 0.79 | 0.53 | 0.32 | 0.42 | 0.81 | 0.68 | 0.68 |
O38 Subcutaneous abatacept in patients with polyarticular-course juvenile idiopathic arthritis and inadequate response to biologic or non-biologic disease-modifying antirheumatic drugs: pharmacokinetics, efficacy and safety
N Ruperto1, DJ Lovell2, N Tzaribachev3, G Vega-Cornejo4, I Louw5, A Berman6, I Calvo7, R Cuttica8, G Horneff9, F Avila-Zapata10, J Anton11, R Cimaz12, E Solau-Gervais13, R Joos14, G Espada15, X Li16, M Nys17, R Wong16, S Banerjee16, A Martini18, HI Brunner2, and For Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology Collaborative Study Group (PRCSG)
1Istituto G. Gaslini Pediatria II Reumatologia, Genova, Italy; 2Cincinnati Children’s Hosp. Medical Center, Cincinnati, OH, USA; 3Pediatric Rheumatology, Bad Bramstedt, Germany; 4CREA (Clinica de Reumatología y Enfermedades Autoinmunes) Hosp. México Americano, Guadalajara, Mexico; 5Panorama Medical Centre, Cape Town, South Africa; 6Univ. Nacional de Tucuman and Centro Médico Privado de Reumatología, Tucuman, Argentina; 7Univ. La Fe, Valencia, Spain; 8Hosp. General de Niños Pedro de Elizalde, Buenos Aires, Argentina; 9Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany; 10Star Medica Hosp., Merida, Mexico; 11Hosp. Sant Joan de Déu, Barcelona, Spain; 12Ospedale Pediatrico Anna Meyer, Firenze, Italy; 13Hôpital de la Milétrie, Poitiers, France; 14University Hosp. Gent, Gent, Belgium; 15Hosp. De Ninos Dr. Ricardo Gutierrez, Buenos Aires, Argentina; 16Bristol-Myers Squibb, Princeton, USA; 17Bristol-Myers Squibb, Braine L’Alleud, Belgium; 18Istituto G. Gaslini Pediatria II Reumatologia and Univ. of Genova, Genova, Italy
Presenting author: N Ruperto
2–5-year cohort (n = 32) | 6–17-year cohort (n = 173) | |
---|---|---|
Deaths | 0 | 0 |
All AEs | 26 (81.3) | 127 (73.4) |
Related AEs | 11 (34.4) | 45 (26.0) |
AEs leading to discontinuation | 0 | 4 (2.3)a
|
SAEs | 0 | 8 (4.6) |
Related SAEs | 0 | 1 (0.6) |
SAEs leading to discontinuation | 0 | 2 (1.2)b
|
AEs of special interest | ||
Malignancies | 0 | 1 (0.6) |
Autoimmune disorders | 0 | 3 (1.7) |
Local injection-site reactions | 0 | 10 (5.8) |
Infections | 22 (68.8) | 90 (52.0) |
O39 Expression of type I and type II interferons is increased in muscle biopsies of juvenile dermatomyositis patients and related to clinical and histological features
Rebecca Nicolai1, Denise Pires Marafon1, Margherita Verardo2, Adele D’Amico2, Luisa Bracci-Laudiero1,3, Fabrizio De Benedetti1, Gian Marco Moneta1
1Division of Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy; 2Unit of Neuromuscular and Neurodegenerative Disease, Department of Neuroscience, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy; 3Institute of translational Pharmacology, CNR, Roma, Italy
Presenting author: Rebecca Nicolai
O40 Next generation sequencing reveals complement deficiencies are the most frequent causes of monogenic lupus in children
Alexandre Belot1, Gillian Rice2, Anne-Laure Mathieu3, Sulliman O. Omarjee3, Brigitte Bader-Meunier4, Thierry Walzer3, Tracy A. Briggs2, James O'Sullivan2, Simon Williams2, Rolando Cimaz5, Eve Smith6, Michael W. Beresford6, Yanick J. Crow7 and GENIAL Investigators, UK JSLE Study Group
1INSERM U1111& Pediatric Rheumatology Unit, Lyon, France; 2Manchester Academic Health Science Centre, Universtity of Manchester, Manchester, United Kingdom; 3INSERM U1111, Lyon, France; 4Pediatric Immunology and Rheumatology, IMAGINE Institute, Necker Hospital, Paris, France, 5Pediatric Rheumatology, Meyer Children Hospital, Firenze University, Firenze, Italy; 6Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom; 7Neuroinflammation, Institut Imagine & Manchester Academic Health Science Centre, Paris, France
Presenting author: Alexandre Belot
Various topics
O41 Prevention and treatment of steroid induced osteopaenia in children and adolescents with rheumatic diseases: the pops study
Madeleine Rooney1, Nick Bishop2, joyce davidson3, Clarissa pilkington4, michael Beresford5, Jacqui Clinch6, Rangaraj Satyapal7, Helen Foster8, Janet Gardner Medwin9, Janet McDonagh10, Sue Wyatt11, and On Behalf of the British Society for Paediatric and Adolescent Rheumatology
1Experimental Medicine, Queens university Belfast, Belfast, UK; 2Academic unit of Child Health, Sheffield University, Sheffield, UK; 3medicine, Royal Hospital for Sick Children (Yorkhill) Glasgow, Glasgow, UK; 4Great Ormond Street Hospital for Children, NHS Trust, London, UK; 5Institute Transnational medicine, University of Liverpool, Liverpool, UK; 6Royal National Hospital for Rheumatic Diseases, Bath, UK; 7Queen's Medical Centre - Nottingham Childrens Hospital, Nottingham, UK; 8Institute of Cellular Medicine, Newcastle University, Newcastle, UK; 9Child Health, University of Glasgow, Glasgow, UK; 10Institute of Inflammation and Repair, University of Manchester, Manchester, UK; 11Rheumatology, Leeds Teaching Hospitals, Leeds, UK
Presenting author: Madeleine Rooney
Variables | Placebo | One alpha | Risedronate | All patients |
---|---|---|---|---|
N | 77 | 71 | 69 | 217 |
Age (years), Mean (SD) | 12.1 (3.5) | 12.1 (3.7) | 12.0 (3.4) | 12.1 (3.5) |
Female, n (%) | 55 (71.4) | 48 (67.6) | 53 (76.8) | 156 (71.9) |
Male, n (%) | 22 (28.6) | 23 (32.4) | 16 (23.2) | 61 (28.1) |
Tanner Score, Median (IQR) | 2 (1 to 4) | 2 (1 to 4) | 2 (1 to 3) | 2 (1 to 4) |
Steroid Dose, n (%) <= 0.2 mg/kg | 37 (48.1) | 30 (42.3) | 32 (46.4) | 99 (45.6) |
Steroid Dose, n (%) >0.2 mg/kg | 40 (52.0) | 41 (57.8) | 37 (53.6) | 118 (54.4) |
Etanercept; Infliximab; Anakinra; Tocilizumab | 8 (10.5%) | 17 (23.9%) | 7 (10.1%) | 32 (14.8%) |
Prior fracture History (Yes), n (%) | 13 (17.1) | 9 (12.7) | 8 (11.6) | 30 (13.89) |
O42 Reduced joint count for ultrasound assessment in juvenile idiopathic arthritis
Valentina Litta Modignani1, Francesco Baldo1, Stefano Lanni2, Alessandro Consolaro2, Angelo Ravelli2, Giovanni Filocamo1
1Dipartimento della Donna, del Bambino o del Neonato, Fondazione IRCCS cà Granda Ospedale Maggiore Policlinico, Milano, Italy; 2IRCCS G Gaslini and Università di Genova, Genova, Italy
Presenting author: Valentina Litta Modignani
Clinical JADAS | |||||||
---|---|---|---|---|---|---|---|
Systemic arthritis | RF + polyarthritis | RF - polyarthritis | Oligoarthritis | Psoriatic arthritis | ER A | Undifferentiated arthritis | |
N 880 | N 346 | N 1914 | N 3516 | N 282 | N 871 | N 457 | |
rUS 6 | 0.77 | 0.82 | 0.77 | 0.73 | 0.70 | 0.65 | 0.69 |
rUS 10 | 0.79 | 0.86 | 0.82 | 0.77 | 0.78 | 0.70 | 0.76 |
Physician’s global assessment | |||||||
rUS 6 | 0.74 | 0.73 | 0.72 | 0.75 | 0.67 | 0.62 | 0.69 |
rUS 10 | 0.75 | 0.76 | 0.76 | 0.77 | 0.74 | 0.65 | 0.75 |
Active joint count | |||||||
rUS 6 | 0.95 | 0.95 | 0.92 | 0.92 | 0.89 | 0.89 | 0.89 |
rUS 10 | 1 | 1 | 1 | 1 | 1 | 1 | 1 |
Poster Session: Autoinflammatory diseases I
P1 The impact of gastrointestinal clinical manifestations in autoinflammatory diseases (AIDS): lessons from the international Eurofever registry
Alessia Omenetti1, Joost Frenkel2, Helen J. Lachmann3, Seza Ozen4, Nicolino Ruperto5, Marco Gattorno5; on behalf of Eurofever Registry
1DINOGMI, University of Genoa, Genoa, Italy; 2University Medical Center Utrecht, Utrecht, Netherlands; 3Royal Free Campus, London, United Kingdom; 4Hacettepe University Children’s Hospital, Ankara, Turkey; 5Giannina Gaslini Institute, Genoa, Italy
Presenting author: Alessia Omenetti
P2 Variable clinical phenotypes and relation of interferon signature with disease activity in ADA 2 deficiency
Antonella Insalaco1, Gianmarco Moneta1, Manuela Pardeo1, Chiara Passarelli2, Camilla Celani1, Virginia Messia1, Fabrizio De Benedetti1
1Division of Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy; 2Divion of Medical Genetics, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy
Presenting author: Antonella Insalaco
P3 Association between familial Mediterranean fever and spondyloarthritis. Clinical characteristics of the French ceremai pediatric cohort
Bilade Cherqaoui1, Linda Rossi-Semerano2, Perrine Dusser2, Véronique Hentgen3, Isabelle Koné-Paut2
1Pediatrics, CHU Estaing, Clermont-Ferrand, France; 2Pediatric Rheumatology, CEREMAI, CHU Bicêtre, France; 3Pediatrics, CEREMAI, CH Versailles, France
Presenting author: Bilade Cherqaoui
P4 Health-related quality of life (HRQOL) of children with PFAPA syndrome
Claire Grimwood1, Perrine Dusser2, Linda Rossi2, Isabelle Kone Paut2, Veronique Hentgen1
1French reference centre for Autoinflammatory diseases, pediatric unit, Versailles Hospital, Le Chesnay, France; 2French reference centre for Autoinflammatory diseases, pediatric unit, CHU Kremlin Bicetre, Kremlin Bicetre, France
Presenting author: Claire Grimwood
Scale | PFAPA patients | FMF controls | P | ||
---|---|---|---|---|---|
Number | Mean | Number | Mean | ||
Pre-school age children (2–7 years) | |||||
Self report : Total score | 15 | 66,6 | 8 | 80,3 | 0,01 |
Proxi-report : Total score | 23 | 68,9 | 14 | 76,9 | 0,12 |
School age children and youth (8–18 years) | |||||
Self report : Total score | 8 | 58 | 13 | 74 | 0,06 |
Proxi-report : Total score | 10 | 57,4 | 13 | 72,3 | 0,08 |
P5 Cryopyrin associated periodic syndromes in Italian patients: evaluation of the rate of NLRP3 mosaicism and search for novel genes
Denise Lasigliè1, Denise Ferrera1, Giulia Amico1, Marco Di Duca1, Roberta Caorsi1, Loredana Lepore2, Antonella Insalaco3, Marco Cattalini4, Laura Obici5, Rita Consolini6, Roberto Ravazzolo1, Alberto Martini1, Isabella Ceccherini1, Ryuta Nishikomori7, Juan Arostegui8, Marco Gattorno1, Silvia Borghini1
1IRCCS G. Gaslini, Genova, Italy; 2IRCCS Burlo Garofalo, Trieste, Italy; 3IRCCS Bambino Gesu’, Roma, Italy; 4Spedali Civili, Brescia, Italy; 5IRCCS San Matteo, Pavia, Italy; 6Università di Pisa, Pisa, Italy; 7Universita’ di Kyoto, Kyoto, Japan; 8Ospedale di Barcelona, Barcelona, Spain
Presenting author: Denise Lasigliè
P6 Monocytes proteomic profile of patients with different autoinflammatory diseases: an approach to identify new biomarkers
Federica Penco1, Andrea Petretto2, Chiara Lavarello2, Elvira Inglese2, Alessia Omenetti3, Martina Finetti3, Claudia Pastorino1, Arinna Bertoni1, Marco Gattorno1
1Laboratorio di Immunologia delle Malattie Reumatiche, Genova, Italy; 2Core Facilities-Laboratorio Proteomica, Genova, Italy; 3Pediatria II, Istituto G. Gaslini, Genova, Italy
Presenting author: Federica Penco
P7 Results of the Eurofever Delphi survey for the classification criteria of PFAPA syndrome
Federica Vanoni1,2, Silvia Federici3, Seza Ozen4, Joost Frenkel5, Helen Lachmann6, Alberto Martini3, Nicola Ruperto3, Marco Gattorno3, Michaël Hofer1, and on behalf of EUROFEVER PROJECT
1Pediatric Rheumatology Unit, CHUV, University of Lausanne, Lausanne, Switzerland; 2Pediatrics, ORBV - Bellinzona, Bellinzona, Switzerland; 3Pediatria II - Reumatologia, Istituto G. Gaslini, Genoa, Italy; 4Department of Pediatric Nephrology and Rheumatology, Hacettepe University, Ankara, Turkey; 5Department of Paediatrics, UMC Utrecht, Utrecht, Netherlands; 6National Amyloidosis Centre, University College Medical School, London, United Kingdom
Presenting author: Federica Vanoni
Rank | Variable | Global Score | Votes (n) |
---|---|---|---|
1 | Regular periodicity | 436 | 56 |
2 | Aphthous stomatitis | 431 | 77 |
3 | Response to steroid | 401 | 66 |
4 | Cervical adenitis | 368 | 72 |
5 | Well being between flares | 299 | 57 |
6 | Pharyngitis (exudative or not) | 288 | 47 |
7 | Increase of acute phase reactants and serum amyloid A during fever episodes | 271 | 44 |
8 | Normal growth/development | 236 | 51 |
9 | Pharingotonsillitis | 228 | 35 |
10 | Periodic fever 3–5 days | 202 | 24 |
11 | Periodic fever 3–6 days | 202 | 23 |
12 | Self limiting episodes | 183 | 35 |
13 | Response to tonsillectomy | 182 | 33 |
14 | Improvement with age | 160 | 40 |
15 | Exclusion cyclic neutropenia/immunodeficiency | 150 | 34 |
16 | Normalization of acute phase reactants in well-being | 146 | 33 |
17 | Recurrence every 3–6 weeks | 145 | 21 |
P8 Safety and efficacy of long-term canakinumab therapy in patients with caps: final results from β-confident registry
J.B. Kuemmerle-Deschner1, H.M. Hoffman2, P.N. Hawkins3, T. van der Poll4, U.A. Walker5, A. Speziale6, Y. Joubert6, H.H. Tilson7
1University Hospital Tuebingen, Tuebingen , Germany; 2University of California, San Diego, CA, USA; 3University College London Medical School, London, United Kingdom; 4Academic Medical Center, University of Amsterdam, Amsterdam, Netherlands; 5University Hospital, Basel, Switzerland; 6Novartis Pharma AG, Basel, Switzerland; 7University of North Carolina, Chapel Hill, NC, USA
Presenting author: J.B. Kuemmerle-Deschner
P9 Development and validation of diagnostic criteria for cryopyrin associated periodic syndromes
Jasmin Kuemmerle-Deschner1, Seza Ozen2, Pascal N. Tyrrell3, Isabelle Koné-Paut4, Raphaela Goldbach-Mansky5, Helen Lachmann6, Norbert Blank7, Hal M. Hoffman8, Elisabeth Weissbarth-Riedel9, Boris Huegle10, Tilmann Kallinich11, Marco Gattorno12, Ahmet Gul13, Nienke M. ter Haar14, Marlen Oswald1, Fatma Dedeoglu15, Susanne M. Benseler16
1Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tuebingen, Tuebingen, Germany; 2Pediatric Nephrology and Rheumatology, Hacettepe University, Ankara, Turkey; 3Department of Medical Imaging, University of Toronto, Toronto, ON, Canada; 4Pediatric Rheumatology, CHU Bicêtre, Le Kremlin Bicêtre, France; 5Translational Autoinflammatory Disease Section, NIAMS/NIH, Bethesda, MD, USA; 6UK National Amyloidosis Centre, University College London Medical School, London, United Kingdom; 7Haematologie, Onkologie und Rheumatologie, Universitaetsklinikum Heidelberg, Heidelberg, Germany; 8Division of Allergy, Immunology and Rheumatology, Department of Pediatrics, UC San Diego/Rady Children’s Hospital, San Diego, CA, USA; 9Kinderrheumatologische Ambulanz, Universitaetsklinikum Eppendorf, Hamburg, Germany; 10German Center for Pediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany, 11Rheumatology, Charité, University Medicine Berlin, Berlin, Germany; 12UO Pediatria 2, G. Gaslini Institute, Genoa, Italy; 13Department of Internal Medicine, Rheumatology Division, Istanbul University, Istanbul, Turkey; 14Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands;15Division of Immunology, Boston Children's Hospital, Boston, MA, USA; 16Rheumatology, Alberta Children's Hospital, University of Calgary, Calgary, AB, Canada
Presenting author: Jasmin Kuemmerle-Deschner
P10 Application of Gaslini Diagnostic Score in Japanese children with periodic fever
Aki Hanaya1, Takako Miyamae2, Manabu Kawamoto2, Yumi Tani1, Takuma Hara2, Yasushi Kawaguchi2, Satoru Nagata1, Hisashi Yamanaka2
1Department of Pediatrics, Tokyo, Japan; 2Institute of Rheumatology, Tokyo Women’s Medical University, Tokyo, Japan
Presenting author: Aki Hanaya
P11 Our experience in the treatment of PAPA syndrome
Almira Ćosićkić1, Fahrija Skokić2, Belkisa Čolić1, Sanimir Suljendić3
1Reumatology, Allergology and Immunology, University Clinical Center, Bosnia and Herzegovina, Tuzla, Bosnia and Herzegovina; 2 Neonatology, University Clinical Center, Bosnia and Herzegovina, Tuzla, Bosnia and Herzegovina; 3Childens Surgery, Childrens Hospital, University Clinical Center, Bosnia and Herzegovina, Tuzla, Bosnia and Herzegovina
Presenting author: Almira Ćosićkić
P12 Enterocolitis and recurrent macrophage activation syndrome in a patient with G786V mutation of NLRC4 gene
Anna Kozlova, Irina Mersiyanova, Mariya Panina, Lily Hachtryan, Vasiliy Burlakov, Elena Raikina, Alexey Maschan, Anna Shcherbina
Immunology, Federal Research and Clinical Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation
Presenting author: Anna Kozlova
P13 Decreased vitamin B12 levels in children with familial Mediterranean fever
Banu Acar1, Meryem Albayrak2
1Department of Pediatric Rheumatology, Kırıkkale University, School of Medicine, Kırıkkale, Turkey; 2Department of Pediatric Hematology, Kırıkkale University, School of Medicine, Kırıkkale, Turkey
Presenting author: Banu Acar
Characteristics | FMF | Control group |
---|---|---|
n (%) | n (%) | |
Age (years) | 8.2 ± 2,72 | 8,5 ± 2,85 |
Arthralgia | 8 (40) | 3 (15) |
Limb pain with exercise / Heel pain | 1 (5)/2 (10) | 0 /0 |
Myalgia / Weakness | 0/0 | 1 (5)/0 |
Tiredness | 4 (20) | 2 (10) |
A loss of appetite, | 3 (15) | 1 (5) |
Mental problems like memory loss, or behavioral changes | 4 (20) | 1 (5) |
Vitamin B12 level (median-pg/ml) | 310.41 | 351.83 |
Folic acid (median-ng/ml) | 8,9 ± 7.2 | 10,3 ± 5.6 |
P14 Canakinumab-treatment in autoinflammatory disease patients younger than 2 years old
Betul Sozeri1, Sezgin Sahin2, Kenan Barut2, Amra Adrovic2, Nese Inan3, Serhan Sevgi3, Ozgur Kasapcopur2
1Pediatric Rheumatology, Erciyes University Faculty of Medicine, Kayseri, Turkey; 2Pediatric Rheumatology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey; 3Novartis Pharmaceutical Corporation, Istanbul, Turkey
Presenting author: Betul Sozeri
P15 Response of pamidronate treatment assessed by whole body magnetic resonance imaging in chronic non-bacterial osteomyelitis
Caroline M. Andreasen1, Anne Grethe Jurik2, Mia B. Glerup3, Christian Høst3, Birgitte T. Mahler3, Ellen-Margrethe Hauge1, Troels Herlin3
1Dept. Rheumatology, Aarhus University Hospital, Aarhus, Denmark; 2Dept. Radiology, Aarhus University Hospital, Aarhus, Denmark; 3Dept. Paediatrics, Aarhus University Hospital, Aarhus, Denmark
Presenting author: Caroline M. Andreasen
P16 Clinical and laboratory characteristics in Marshall syndrome
Cecilia Lazea1, Laura Damian2, Calin Lazar1, Rodica Manasia1
1Pediatrics, University of Medicine and Pharmacy Cluj-Napoca, Cluj-Napoca, Romania; 2Rheumatology, County Emergency Hospital, Cluj-Napoca, Romania
Presenting author: Cecilia Lazea
P17 Effective treatment of severe juvenile pustular psoriasis with cyclosporine and etanercept: a case report utilizing autoinflammatory diseases activity index (AIDAI) to document treatment response
Chloe M. Stephenson1, Vimal Prajapati1, Paivi M. Miettunen2
1University of Calgary, Calgary, Canada; 2Paediatrics, University of Calgary, Calgary, Canada
Presenting author: Chloe M. Stephenson
P18 The prevelance of Fabry disease in familial Mediterranean fever children from Turkey
Dilek Yılmaz1, Yavuz Tokgöz2, Yasin Bulut3, Harun Çakmak4, Ferah Sönmez1
1Pediatric Nephrology, Adnan Menderes University Faculty of Medicine, Aydın, Turkey; 2Pediatric Gastroenterology, Adnan Menderes University Faculty of Medicine, Aydın, Turkey; 3Pediatrcis, Adnan Menderes University Faculty of Medicine, Aydın, Turkey; 4Ophtalmology, Adnan Menderes University Faculty of Medicine, Aydın, Turkey
Presenting author: Dilek Yılmaz
Variables | Patients (n = 76) |
---|---|
Female (n, %) | 43 (56.5) |
Male (n, %) | 33 (43.5) |
Age (mean ± SD), years | 12.3 ± 3.9 |
Alpha-galactosidase A (mean ± SD), μmol/l/h | 2.95 ± 1.09 |
Clinical parameters | |
Fever (%) | 68.4 |
Abdominal pain (%) | 86.6 |
Chest pain (%) | 30.3 |
Arthritis (%) | 72.4 |
Family history of FMF (%) | 76.3 |
P19 Is R202Q alteration of MEFV gene a disease-causing mutation?
Elif Comak1, Gülşah Kaya Aksoy1, Mustafa Koyun1, Sema Akman1, Yunus Arıkan2, Ender Terzioğlu3, Osman Nidai Özdeş2, İbrahim Keser2, Hüseyin Koçak4, Ayşen Bingöl5, Aygen Yılmaz6, Reha Artan6
1Pediatric Nephrology - Rheumatology, Akdeniz University, Antalya, Turkey; 2Medical Genetics, Akdeniz University, Antalya, Turkey; 3Rheumatology, Akdeniz University, Antalya, Turkey; 4Nephrology, Akdeniz University, Antalya, Turkey; 5Immunology, Akdeniz University, Antalya, Turkey; 6Pediatric Gastroenterology, Hepatology & Nutrition, Akdeniz University, Antalya, Turkey
Presenting author: Elif Comak
P20 Pharmacokinetics and pharmacodynamics of canakinumab in patients with periodic fever syndromes (colchicine-resistant FMF, HIDS/MKD and TRAPS): results from a phase III pivotal umbrella trial
F. De Benedetti1, J. Anton2, M. Gattorno3, H. Lachmann4, I. Kone-Paut5, S. Ozen6, J. Frenkel7, A. Simon8, A. Zeft9, E. Ben-Chetrit10, H.M. Hoffman11, Y. Joubert12, K. Lheritier12, A. Speziale12, J. Guido12, X. Xu13
1IRCCS Ospedale Pediatrico Bambino Gesú, Rome, Italy; 2Hospital Sant Joan de Déu, Barcelona, Spain; 3Pediatric Rheumatology, G. Gaslini Institute, Genoa, Italy; 4UK National Amyloidosis Centre, University College London Medical School, London, United Kingdom; 5Hôpital Kremlin Bicetre, University of Paris SUD, Paris, France; 6 Department of Pediatrics, Hacettepe University, Ankara, Turkey; 7University Medical Center Utrecht, Utrecht, Netherlands; 8Radboud University Medical Centre, Nijmegen, Netherlands; 9Pediatrics Rheumatology, Cleveland Clinic, Cleveland, OH, USA; 10Rheumatology Unit, Hadassah—Hebrew University Medical Center, Jerusalem, Israel; 11University of California, San Diego, La Jolla, CA, USA; 12Novartis Pharma AG, Basel, Switzerland; 13Novartis Pharmaceuticals Corporation, East Hanover, NJ, USA
Presenting author: F. De Benedetti
P21 Gene mutation of familial Mediterranean fever (FMF) registry in Iran
Fatemeh F. Mehregan1, Vahid Ziaee2, Mohammad H. Moradinejad2
1Pediatric Rheumatology, Shahid Beheshti University of Medical Sciences, Tehran, Iran, Islamic Republic Of; 2Pediatric Rheumatology, Tehran University of Medical Science, Tehran, Iran, Islamic Republic Of
Presenting author: Fatemeh F. Mehregan
P22 Observational clinical survey and investigation of autoinflammatory candidate genes role in an Italian cohort of pediatric patients with chronic non-bacterial osteomyelitis
Giovanna Ferrara1, Serena Pastore2, Antonella Insalaco3, Manuela Pardeo3, Alberto Tommasini2, Francesco La Torre4, Clotilde Alizzi5, Rolando Cimaz6, Martina Finetti7, Marco Gattorno7, Pio D’Adamo1, Andrea Taddio1,2
1University of Trieste, Trieste, Italy; 2Institute of Child and Maternal Health – IRCCS “Burlo Garofolo”, Trieste, Italy; 3IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy; 4Antonio Perrino Hospital, Brindisi, Italy; 5Ospedale Pediatrico G. Di Cristina di Palermo, Palermo, Italy; 6Anna Meyer Children Hospital, Florence, Italy; 7UOC Pediatria 2, Istituto Gaslini, Genova, Italy
Presenting author: Giovanna Ferrara
P23 Effect of canakinumab on health-related quality of life in patients with periodic fever syndromes
H. Lachmann1, A. Simon2, J. Anton3, M. Gattorno4, I. Kone-Paut5, S. Ozen6, J. Frenkel7, E. Ben-Chetrit8, H. Hoffman9, A. Zeft10, Y. Joubert11, K. Lheritier11, A. Speziale11, G. Junge11, J. Gregson11, F. De Benedetti12
1UK National Amyloidosis Centre, University College London Medical School, London, United Kingdom; 2Radboud University Medical Centre, Nijmegen, Netherlands; 3Hospital Sant Joan de Déu, Barcelona, Spain; 4Pediatric Rheumatology, G. Gaslini Institute, Genoa, Italy; 5Hôpital Kremlin Bicetre, University of Paris SUD, Paris, France; 6Hacettepe University Children’s Hospital, Ankara, Turkey; 7University Medical Center Utrecht, Utrecht, Netherlands; 8Rheumatology Unit, Hadassah—Hebrew University Medical Center, Jerusalem, Israel; 9University of California, La Jolla, USA; 10Pediatrics Rheumatology, Cleveland Clinic, Cleveland, OH, USA; 11Novartis Pharma AG, Basel, Switzerland; 12IRCCS Ospedale Pediatrico Bambino Gesú, Rome, Italy
Presenting author: H. Lachmann
Outcome measures | Mean change from baseline (n/N)a,c
| |||||
---|---|---|---|---|---|---|
crFMF | HIDS/MKD | TRAPS | ||||
Week 5 | Week 16 | Week 5 | Week 16 | Week 5 | Week 16 | |
SF-12 PCS | 7.9 (29/30) | 9.55 (30/31) | 13.81 (15/15) | 13.81 (14/14) | 9.63 (16/17) | 11.64 (13/14) |
SF-12 MCS | 4.83 (29/30) | 4.27 (30/31) | 6.41 (15/15) | 8.14 (14/14) | 5.65 (16/17) | 5.51 (13/14) |
CHQ-PF50 PhS | 13.2 (21/24) | 20.1 (18/21) | 5.5 (32/34) | 9.9 (27/29) | 7.4 (16/18) | 14.9 (13/14) |
CHQ-PF50 PsS | 4.1 (21/24) | 7.2 (18/21) | 1.8b (32/34) | 5.2 (27/29) | 0.9b (16/18) | 1.2b (13/14) |
P24 Recurrent hyperbilirubinemia as a rare feature of familial Mediterranean fever
Hasmik Sargsyan
Yerevan State Medical University, Yerevan, Armenia
P25 Is the Gilbert syndrome coincidence of familial Mediterranean fever: case report
Hasmik Sargsyan
Yerevan State Medical University, Yerevan, Armenia
P26 Evaluation of biopsychosocial aspects of patients with juvenile Behçet’s disease: a qualitative study
Hulya Zengin1, Berna E. Fidanci1, Cagla Kaymakamgil1, Dilek Konukbay1, Dogan Simsek2, Ezgi D. Batu3, Dilek Yildiz1, Faysal Gok4, Seza Ozen3, Erkan Demirkaya5
1Child Health Nursing, Gülhane Military Medical Academy, Ankara, Turkey; 2Pediatric Rheumatology, Gülhane Military Medical Academy, Ankara, Turkey; 3Pediatric Rheumatology, Hacettepe University, Ankara, Turkey; 4Pediatric Nephrology, Gülhane Military Medical Academy, Ankara, Turkey; 5Pediatric Rheumatology, FAVOR, Ankara, Turkey
Presenting author: Hulya Zengin
P27 Comparison of neutrophilic activation and secretion of cytokines derived from patients with familial Mediterranean fever and other acute and chronic inflammatory diseases
Iris Stoler1, Judith Freytag1, Banu Orak1, Christine Seib1, Lars Esmann2, Eva Seipelt3, Faekah Gohar4, Dirk Foell4, Helmut Wittkowski4, Tilmann Kallinich1
1Department of Pediatric Rheumatology, Charité Berlin, Berlin, Germany; 2Charité Berlin, Berlin, Germany; 3Immanuel Berlin Buch, Berlin, Germany; 4University Hospital Muenster, Albert-Schweitzer-Campus 1, Gebäude W30, Münster, Germany
Presenting author: Iris Stoler
P28 Nail fold capillary abnormality and insulin resistance in children with familial Mediterranean fever: is there any relationship between vascular changes and insulin resistance?
Ismail Dursun1, Sebahat Tulpar1, Sibel Yel1, Demet Kartal2, Murat Borlu2, Funda Bastug1, Hakan Poyrazoglu1, Zubeyde Gunduz1, Kader Kose3, Mehmet E. Yuksel4, Abdullah Calıskan4, Ahmet B. Cekgeloglu4, Ruhan Dusunsel1
1Pediatric Nephrology, Erciyes University, Faculty of Medicine, Kayseri, Turkey; 2Dermatology and Venereology, Erciyes University, Faculty of Medicine, Kayseri, Turkey; 3Biochemistry, Erciyes University, Faculty of Medicine, Kayseri, Turkey; 4Department of Biomedical Engineering, Faculty of Engineering, Kayseri, Turkey
Presenting author: Ismail Dursun
P29 Chronic recurrent multifocal osteomyelitis (CRMO): a single centre experience
Katerina Bouchalova1,2, Jana Franova3, Marcel Schuller3, Marie Macku3
1Dpt. of Pediatrics, Palacky University and Faculty Hospital, Olomouc, Czech Republic; 2Institute of Molecular and Translational Medicine, Palacky University and Faculty Hospital, Olomouc, Czech Republic; 3Pediatric Department, University Hospital, Brno, Czech Republic
Presenting author: Katerina Bouchalova
P30 A case of R92Q positive traps patient with aseptic meningitis and intracranial hypertension
Katerina Theodoropoulou1, Raffaella Carlomagno1, Annette von Scheven-Gête1, Claudia Poloni2, Michael Hofer1
1Pediatric Rheumatology; 2Pediatric Neurology, CHUV, Lausanne, Switzerland
Presenting author: Katerina Theodoropoulou
Poster Session: Bone in rheumatic diseases
P31 Chronic recurrent multifocal osteitis and Coxiella Burneti infection mimicking adult-onset Still’s disease
Laura O. Damian1, Dan Cosma2, Amanda Radulescu3, Dan Vasilescu4, Liliana Rogojan5, Calin Lazar6, Simona Rednic7, Mihaela Lupse3
1Rheumatology, Emergency Clinical County Hospital Cluj, Cluj-Napoca, Romania; 2Pediatric Surgery, University of Medicine and Pharmacy Cluj, Cluj-Napoca, Romania; 3Infectious Diseases, University of Medicine and Pharmacy Cluj, Cluj-Napoca, Romania; 4Radiology, University of Medicine and Pharmacy Cluj, Cluj-Napoca, Romania; 5Pathology, Emergency Clinical County Hospital Cluj, Cluj-Napoca, Romania; 61st Pediatric, University of Medicine and Pharmacy Cluj, Cluj-Napoca, Romania; 7Rheumatology, University of Medicine and Pharmacy Cluj, Cluj-Napoca, Romania
Presenting author: Laura O. Damian
P32 Limping as presenting symptom of osteopetrosis in children
Lien De Somer1, Pierre Moens2, Carine Wouters1
1Department of Pediatric Rheumatology, UZ Leuven, Leuven, Belgium; 2Department of Orthopedic Surgery, UZ Leuven, Leuven, Belgium
Presenting author: Lien De Somer
P33 Markers of bone metabolism: are they useful to predict risk of low bone mineral density in juvenile idiopathic arthritis paediatric patients?
Rocio Galindo Zavala1, Laura Martín Pedraz2, Esmeralda Núñez Cuadros1, Gisela Díaz-Cordovés Rego3, Antonio L. Urda Cardona2
1Paediatric Rheumatology, Universitary Regional Malaga Hospital, Malaga, Spain; 2Paediatrics, Universitary Regional Malaga Hospital, Malaga, Spain; 3Rheumatology, Universitary Regional Malaga Hospital, Malaga, Spain
Presenting author: Rocio Galindo Zavala
Patient’s characteristics (N = 94) | ||
Gender (Male), n (%) | 33 (35,1) | |
Age (years), median (IR) | 11,42 (8,58–13,67) | |
Z-score BMD adjusted for height, mean (±SD) | 0,04 (±0,94) | |
DISEASE CHARACTERISTICS (N = 94) | ||
JIA subtype, n (%) | Systemic | 10 (10,6) |
Oligoarticular | 53 (56,4) | |
Polyarticular | 22 (23,4) | |
Others | 9 (9,6) | |
Disease duration (years), median (IR) | 6,12 (3,22–9,10) | |
RECEIVED TREATMENTS (N = 94)* | ||
Systemic GC, n (%) | 84(89,4) | |
Synthetic/biological DMARDs treatment, n (%) | 42 (44,7)/29 (30.9) |
P34 Low bone mineral density in juvenile idiopathic arthritis: prevalence and related factors
Rocio Galindo Zavala1, Laura Martín Pedraz2, Esmeralda Núñez Cuadros3, Gisela Díaz-Cordovés Rego1, Antonio L. Urda Cardona4
1Paediatric Rheumatology, University Regional Hospital of Malaga, Malaga, Spain; 2Paediatrics, Malaga University Regional Hospital, Malaga, Spain; 3Paediatric Rheumatology, Malaga University Regional Hospital, Malaga, Spain; 4Paediatrics, University Regional Hospital of Malaga, Malaga, Spain
Presenting author: Rocio Galindo Zavala
Patient’s characteristics (N = 94) | ||
Gender (Male), n (%) | 33 (35,1) | |
Age (years), median (IR) | 11,42 (8,58–13,67) | |
Z-score BMD adjusted for height, mean (±SD) | 0,04 (±0,94) | |
Disease characteristics (N = 94) | ||
JIA subtype, n (%) | Systemic | 10 (10,6) |
Oligoarticular | 53 (56,4) | |
Polyarticular | 22 (23,4) | |
Others | 9 (9,6) | |
Received treatments (N = 94)* | ||
Systemic GC, n (%) | 84(89,4) | |
Synthetic/biological DMARDs treatment, n (%) | 42 (44,7)/29 (30.9) |
P35 Evaluation of bone metabolism in juvenile idiopathic arthritis: biochemical bone turnover markers and bone density parameters
Ilaria Dal Forno, Sara Pieropan, Ombretta Viapiana, Davide Gatti, Gloria Dallagiacoma, Paola Caramaschi, Domenico Biasi
Rheumatology, Policlinico G.B.Rossi, Verona, Italy
Presenting author: Sara Pieropan
Poster Session: Imaging
P36 Pediatric musculoskeletal ultrasound – age- and sex-related normal B-mode findings of the achilles tendon
Daniel Windschall1, Ralf Trauzeddel2, Hartwig Lehmann3, Gerd Ganser4, Rainer Berendes5, Maria Haller6, Manuela Krumrey-Langkammerer7, Antje Nimtz-Talaska8, Philipp Schoof9, Ralf Felix Trauzeddel10, Christine Nirschl1
1Asklepios Hospital Weissenfels, Weissenfels, Germany; 2Helios Kliniken Berlin Buch, Berlin, Germany; 3University of Giessen and Marburg, Giessen, Germany; 4St. Joseph Stift, Sendenhorst, Germany; 5Kinderkrankenhaus St. Marien, Landshut, Germany; 6Kinderärztliche Praxis Gundelfingen, Gundelfingen, Germany; 7German Center for Pediatric Rheumatology, Garmisch-Partenkirchen, Germany; 8Kinderärztliche Praxis Frankfurt/Oder, Frankfurt/Oder, Germany; 9Kinderärztliche Praxis, München, Germany; 10Department of Anesthesiology and Intensive Care Medicine, Campus Charité Mitte and Campus Virchow-Klinikum, Berlin, Germany
Presenting author: Daniel Windschall
P37 Usefulness of ultrasound joint score in the assessment of clinical activity in children with juvenile idiopathic arthritis
Estefania Quesada-Masachs1, Carla Aguilar Blancafort2, Sara Marsal Barril3, Consuelo Modesto Caballero4
1Paediatric Rheumartology Unit, Vall d’Hebron University Hospital, Barcelona, Spain; 2Clinical Research Support Unit, Vall d’Hebron Research Institute, Barcelona, Spain; 3Rheumatology, Vall d’Hebron University Hospital, Barcelona, Spain; 4Paediatric Rheumatology Unit, Vall d’Hebron University Hospital, Barcelona, Spain
Presenting author: Estefania Quesada-Masachs
P38 The prevalence of sacroiliitis assessed by magnetic resonance imaging in a pediatric population with inflammatory bowel disease
Francisca Aguiar1, Rita Fonseca1, Duarte Alves2, Ana Vieira3, Alberto Vieira2,3, Jorge A. Dias2,4, Iva Brito2,5
1Rheumatology, Centro Hospitalar São João, Oporto, Portugal; 2Faculty of Medicine of Porto University, Porto, Portugal; 3Radiology, Centro Hospitalar São João, Porto, Portugal; 4Pediatric Gastroenterology, Centro Hospitalar São João, Porto, Portugal; 5Pediatric Rheumatology Unit, Centro Hospitalar São João, Porto, Portugal
Presenting author: Francisca Aguiar
P39 Specificity and sensitivity of salivary glands ultrasonography in juvenile Sjőgren’s syndrome
Gordana Susic1, Vera Milic1,2, Goran Radunovic1,2, Ivan Boricic2,3
1Institute of Rheumatology, University of Belgrade, Belgrade, Serbia; 2Medical faculty, University of Belgrade, Belgrade, Serbia; 3Institute of pathology, Belgrade, Serbia
Presenting author: Gordana Susic
P40 Conventional radiography in juvenile idiopathic arthritis: joined recommendations from the French societies for rheumatology, radiology, paediatric rheumatology
Pauline Marteau1, Catherine Adamsbaum2, Linda Rossi-Semerano3, Michel De Bandt4, Irène Lemelle5, Chantal Deslandre6, Tu Anh Tran7, Anne Lohse8, Elisabeth Solau-Gervais9, Pascal Pillet10, Brigitte Bader-Meunier11, Julien Wipff12, Cécile Gaujoux-Viala13, Sylvain Breton14, Valérie Devauchelle-Pensec15
1Rheumatology, CHU Cavale Blanche, Brest, France; 2Paediatric Radiology, AP-HP Bicêtre, France; 3Paediatric Rheumatology, AP-HP, Hôpital Bicêtre, Paris, France; 4Rheumatology, CHU Martinique, Fort de France, France; 5Rheumatology, University Hospital, Nancy, France; 6Paediatrics, Hôpital Robert Debré, Paris, France; 7Paediatrics, University Hospital, Nîmes, France; 8Rheumatology, Hospital Centre of Belfort Montbeliard, Belfort, France; 9Rheumatology, University Hospital of Poitiers, Poitiers, France; 10Paediatrics, Hôpital Pellegrin, Bordeaux, Bordeaux, France; 11Paediatric Rheumatology, Hôpital Necker, Paris, France; 12Rheumatology, Hôpital Cochin, Paris, France; 13Rheumatology, University Hospital, Nîmes, France; 14Paediatric Radiology, Hôpital Necker, Paris, France; 15Rheumatology, Hôpital Cavale Blanche, Brest, France
Presenting author: Pauline Marteau
P41 Monitoring disease severity in experimental arthritis by imaging of phagocyte migration in vivo
Sandra Gran1, Olesja Fehler1, Stefanie Zenker1, Michael Schäfers2, Johannes Roth1, Thomas Vogl1
1Institute of Immunology, Münster, Germany; 2European Institute for Molecular Imaging, Münster, Germany
Presenting author: Sandra Gran
P42 Impact of S100A12 proteins quantification and MSUS in treatment management of JIA patients with complete remission on treatment; a pilot study in 13 patients
Severine Guillaume Czitrom1, Dirk Foell2, Dirk Holzinger2
1Service de Medecine des Adolescents, Chu de Bicetre, Le Kremlin Bicetre Cedex, France; 2Klinik für Pädiatrische Rheumatologie, Universitätsklinikum Münster, Münster, Germany
Presenting author: Severine Guillaume Czitrom
P43 Sublinical synovitis on us in patients with new-onset juvenile idiopathic arthritis does not predict early appearance of clinical disease except for the ankle joint
Stefano Lanni1, EH Pieter Van Dijkhuizen1,2, Silvia Magni Manzoni3, Denise Pires Marafon3, Francesca Magnaguagno1, Laura Tanturri de Horatio3, Nienke M Ter Haar2, Annemieke S Littooij2, Sebastiaan J Vastert2, Fabrizio De Benedetti3, Angelo Ravelli1,4, Alberto Martini1, Clara Malattia1,4
1Istituto Giannina Gaslini, Genova, Italy; 2University Medical Center Utrecht, Utrecht, Netherlands; 3Ospedale Pediatrico Bambino Gesù, Roma, Italy; 4Università degli Studi di Genova, Genova, Italy
Presenting author: Stefano Lanni
P44 Musculoskeletal ultrasound usefulness in the diagnosis and treatment in pediatric rheumatology
Vitor A. Teixeira1, Raquel Campanilho-Marques1,2, Ana F. Mourão2,3, Filipa O. Ramos1,2,3, Manuela Costa1
1Serviço de Reumatologia e Doenças Ósseas Metabólicas, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon, Portugal; 2Unidade de Reumatologia Pediátrica, Serviço de Reumatologia e Departamento de Pediatria, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon, Portugal; 3Instituto de Medicina Molecular, Faculdade de Medicina de Lisboa., Lisbon, Portugal
Presenting author: Vitor A. Teixeira
P45 MRI imaging of the temperomandibular joint in children with JIA: a 3 year retrospective study
Wafa A. Madan1, Orla G. Killeen2
1National Centre for Paediatric Rheumatology, Our Lady’s Childrens Hospital, Dublin, Ireland; 2National Centre for Paediatric Rheumatology, Our Lady’s Children Hospital Crumlin, Dublin, Ireland
Presenting author: Wafa A. Madan
P46 Temporomandibular signs ands symptons associated with MRI findings in patients with juvenil idiopathic arthritis
Adriana Rodriguez Vidal, Diana Sueiro Delgado, Maria Isabel Gonzalez Fernandez, Berta Lopez Montesinos, Inmaculada Calvo Penades
Pediatric Rheumatology, Hospital La Fe, Valencia, Spain
Presenting author: Adriana Rodriguez Vidal
P47 CACP-syndrome in pediatric rheumatology. Clinical and instrumental diagnostic criteria
Aleksey Kozhevnikov1, Nina Pozdeeva1, Mikhail Konev1, Evgeniy Melchenko2, Vladimir Kenis2, Gennadiy Novik3
1Children’s Orthopedic and Rheumatologic Department., FSBI “The Turner Scientific and Research Institute for Children’s Orthopedics” under the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian Federation; 2Department of Foot Pathology, Neuroothopedics and Systemic Diseases, FSBI “The Turner Scientific and Research Institute for Children’s Orthopedics” under the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian Federation; 3Pediatric department n.a. I. M. Voroncov, Saint-Petersburg State Pediatric Medical University under the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian Federation
Presenting author: Aleksey Kozhevnikov
P48 Comparing articular cartilage thickness in JIA patients and healthy controls by ultrasonography
Betul Sozeri, Aysenur Pac Kısaarslan, Zubeyde Gunduz, Hakan Poyrazoglu, Ruhan Dusunsel
Pediatric Rheumatology, Erciyes University, Faculty of Medicine, Kayseri, Turkey
Presenting author: Betul Sozeri
P49 The comparisons between thermography and ultrasonography with physical examination for wrist joint assessment in juvenile idiopathic arthritis
Butsabong Lerkvaleekul1, Suphaneewan Jaovisidha2, Witaya Sungkarat2, Niyata Chitrapazt2, Praman Fuangfa2, Thumanoon Ruangchaijatuporn2, Soamarat Vilaiyuk1
1Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand, 2Radiology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
Presenting author: Butsabong Lerkvaleekul
P50 Radiographic evaluation of joint space width compared to cartilage thickness as assessed by ultrasonography in MCP, PIP, and knee joints of children with juvenile idiopathic arthritis
Dan Ø. Pradsgaard1, Arne Hørlyck2, Anne H. Spannow1, Carsten W. Heuck1, Troels Herlin1
1Pediatrics, Aarhus University Hospital, Aarhus, Denmark; 2Radiology, Aarhus University Hospital, Aarhus, Denmark
P51 Study test match between musculoskeletal ultrasound and physical examination with acute phase reactants (C reactive protein and erythrocyte sedimentation rate) for subclinical synovitis detection in mexican with juvenile idiopathic arthritis
Talia Diaz1, Fernando Garcia2, Lorenia De La Cruz3, Nadina Rubio2
1Paediatrics, Hospital San José Tec de Monterrey, Monterrey, Mexico; 2Paediatric Rheumatology, Hospital Universitario “Dr. José Eleuterio González”, Monterrey, Mexico; 3Rheumatology, Hospital Universitario “Dr. José Eleuterio González”, Monterrey, Mexico
Presenting author: Fernando Garcia
P52 A new tool of disease activity evaluation through-out power doppler ultrasonography and serum vascular markers in juvenile idiopathic arthritis
Joanna Świdrowska-Jaros, Elzbieta Smolewska
Department of Pediatric Cardiology and Rheumatology, Medical University of Lodz, Lodz, Poland
Presenting author: Joanna Świdrowska-Jaros
P53 Thermal point of care diagnostic tool for measurement of joint inflammation
Mirta Lamot1, Lovro Lamot1,2, Mandica Vidovic1, Edi Paleka Bosak1, Ivana Rados1, Miroslav Harjacek1
1Clinical Hospital Center Sestre Milosrdnice, Zagreb, Croatia; 2University of Zagreb School of Medicine, Zagreb, Croatia
Presenting author: Lovro Lamot
P54 Quantification of dynamic contrast enhanced MRI (DCE-MRI) examinations in juvenile idiopathic arthritis
Nikolay Tzaribachev1, Polymnia Louka2, Romiesa Hagoug2, Chiara Trentin2, Olga Kubassova2, Mark Hinton2, Mikael Boesen3
1PRI - Pediatric Rheumatology Research Institute, Bad Bramstedt, Germany; 2Image Analysis, London, United Kingdom; 3Department of Radiology, Bispebjerg and Frederiksberg Hospital Copenhagen University, Copenhagen, Denmark
Presenting author: Nikolay Tzaribachev
ALL MCP (2-5) -DEMRIQ inflammation | Global clinical scores-AJ | Outcome | ||||||
---|---|---|---|---|---|---|---|---|
Patient | Baseline | 3 mo | 6 mo | Pearson’s correlation | Baseline | 3 mo | 6 mo | |
1 | 117,83 | 151,14 | 135,66 | Moderate (+) | 13 | 5 | 7 | Switch DMARD |
2 | 83,16 | 28,96 | 64,89 | Very strong (-) | 8 | 3 | 0 | Continue DMARD |
3 | 99,28 | 46,16 | 26,35 | Very strong (-) | 9 | 2 | 11 | Improved later |
4 | 38,28 | 104,71 | 87,86 | Strong (+) | 12 | 6 | 0 | Flare at 6 mo |
5 | 113,36 | 102,6 | 118,39 | Moderate (+) | 12 | 4 | 0 | Flare at 6 mo |
6 | 29,28 | 39,09 | 58,12 | Weak | 8 | 6 | 0 | Flare at 2 mo |
7 | 38,1 | 19,59 | 56,47 | Strong (+) | 13 | 0 | 0 | Flare at 3 mo |
8 | 50,42 | 96,76 | 74,75 | Moderate (-) | 7 | 2 | 0 | Flare at 3 mo |
P55 Value of magnetocardiography for diagnosis of myocardial damage in children with rheumatic diseases
Olena A. Oshlianska1, Illya A. Chaikovsky2, G. Mjasnikov3, A. Kazmirchyk3
1State Institute of Pediatrics Obstetrics and Gynecology NAMS, Kyiv, Ukraine; 2V.M. Glushkov Institute of Cybernetics of NAS of Ukraine, Kyiv, Ukraine; 3National Military Medical Center of Ukraine, Kyiv, Ukraine
Presenting author: Olena A. Oshlianska
P56 Early detection and follow-up of morphological alterations of temporomandibular joint in juvenile idiopathic arthritis: high-resolution ultrasound
Umberto Garagiola, Irene Borzani, Paolo Cressoni, Fabrizia Corona, Eszter Dzsida, Giampietro Farronato
Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milano, Italy
P57 Correlation between maxillary sinusitis and juvenile idiopathic arthritis: cone beam CT study
Umberto Garagiola1, Paolo Cressoni1, Fabrizia Corona1, Antonella Petaccia1, Eszter Dzsida1, Giampietro Farronato1
1Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milan, Italy
Presenting author: Umberto Garagiola
Poster Session: Immunodeficiency and infection related arthritis
P58 Attending to warning signs of primary immunodeficiency diseases in rheumatology practice: focus on juvenile idiopathic arthritis-like disease
Alenka Gagro, Agneza Marija Pasini, Goran Roic, Ozren Vrdoljak, Lucija Lujic, Matija Zutelija-Fattorini
Children’s Hospital Zagreb, Zagreb, Croatia
Presenting author: Alenka Gagro
PID (n) | Gender | ANA | RF | HLA association | Diagnosis of PID prior to arthritis (yrs) | Age at arthritis onset (yrs) |
---|---|---|---|---|---|---|
sIgAD (2) | F | + | - | - | No | 2 |
F | - | - | - | No | 4 | |
AT (1) | M | - | - | - | Yes/1 | 14 |
APECED (1) | M | - | - | - | No | 6 |
ALPS (1) | F | - | - | - | Yes/13 | 15 |
FMF (1) | F | - | - | - | Yes/11 | 12 |
P59 Familial Aicardi–Goutières Syndrome with SAMDH1 mutation –phenotype variation in 2 sisters from South Africa
Monika M. Esser1, Deepthi R. Abraham2, Craig Kinnear2, Glenda Durrheim2, Mike Urban2, Eileen Hoal2, Yanick Crow3
1Pathology, Stellenbosch University, National Health Laboratory Services, Cape Town, South Africa; 2Stellenbosch University, Cape Town, South Africa; 3University of Manchester, Manchester, United Kingdom
Presenting author: Monika M. Esser
P60 Aseptic arthritis in children associated with primary immune deficiencies
Olena A. Oshlianska, Victoria B. Nikolayenko
Connective children diseases in children, State Institute of Pediatrics Obstetrics and Gynecology NAMS of Ukraine, Kyiv, Ukraine
Presenting author: Olena A. Oshlianska
P61 Retrospective analysis of laboratory data and treatments of 43 adult patients followed up with the diagnoses of acute rheumatic fever (ARF) and post-streptococcal reactive arthritis (PSRA)
Kubilay Şahin1, Yasar Karaaslan2
1Rheumatology, Mersin State Hospital; Keçiören Education and Research Hospital, Ankara, Turkey; 2Rheumatology, Hitit University Medical Faculty, Ankara Numune Education and Research Hospital, Ankara, Turkey
Presenting author: Yasar Karaaslan
Poster Session: JIA (oligo, poly, psoriatic) I
P62 Musculoskeletal symptoms at the onset of pediatric tumors and predictive features in the differential diagnosis with juvenile idiopatic arthritis: preliminary analysis of a multicenter, prospective, observational study
Adele Civino1, Giovanni Alighieri1, Sergio Davì2, Roberto Rondelli3, Silvana Martino4, Giovanni Filocamo5, Andrea Magnolato6, Francesca Ricci7, Romina Gallizzi8, Alma Olivieri9, Valeria Gerloni10, Bianca Lattanzi11, Francesca Soscia12, Alessandro De Fanti13, Silvia Magni Manzoni14, Stefania Citiso1, Lorenzo Quartulli1, Francesco La Torre15, Donato Rigante16, Maria Cristina Maggio17, Manuela Marsili18, Maria Antonietta Pelagatti19, Valentino Conter20, Franca Fagioli21, Loredana Lepore6, Andrea Pession3, Angelo Ravelli2
1Pediatric Rheumatology, Tricase (LE), Italy; 2Pediatric Rheumatology, Genova, Italy; 3Pediatric Oncology, Bologna, Italy; 4Pediatric Rheumatology, Torino, Italy; 5Pediatric Rheumatology, Milano - De Marchi, Italy; 6Pediatric Rheumatology, Trieste, Italy; 7Pediatric Rheumatology, Brescia, Italy; 8Pediatric Rheumatology, Messina, Italy; 9Pediatric Rheumatology, Napoli, Italy; 10Pediatric Rheumatology, Milano - G. Pini, Italy; 11Pediatric Rheumatology, Ancona, Italy; 12Pediatric Rheumatology, Orvieto, Italy; 13Pediatric Rheumatology, Reggio Emilia, Italy; 14Pediatric Rheumatology, Roma - Bambino Gesù, Italy; 15Pediatric Rheumatology, Brindisi, Italy; 16Pediatric Rheumatology, Roma - Cattolica, Italy; 17Pediatric Rheumatology, Palermo, Italy; 18Pediatric Rheumatology, Chieti, Italy; 19Pediatric Rheumatology, Monza, Italy; 20Pediatric Oncology, Monza, Italy; 21Pediatric Oncology, Torino, Italy
Presenting author: Adele Civino
Tumors with joint involvement (60 pts) | JIA (147 pts) |
p-value | |||
---|---|---|---|---|---|
n° | % | n° | % | ||
Arthritis | 23 | 38,3 | 107 | 72,8 | 0.000 |
Bone pain | 24 | 40,0 | 4 | 2,7 | 0.000 |
Nighttime pain | 7 | 11,7 | 6 | 4,1 | 0.057 |
Pain of the spine | 15 | 25,0 | 12 | 8,2 | 0.002 |
Unproportioned pain | 8 | 13,3 | 2 | 1,4 | 0.001 |
Morning stiffness | 1 | 1,7 | 80 | 54,4 | 0.000 |
Limp | 25 | 41,7 | 89 | 60,5 | 0.014 |
Only musculoskeletal symptoms | 16 | 26,7 | 116 | 78,9 | 0.000 |
P63 Differences in disease phenotype, management and outcomes of children with juvenile idiopathic arthritis throughout the world – analysis of 8,325 patients enrolled in the EPOCA study
Alessandro Consolaro1, Nicolino Ruperto2, Marco Garrone2, Mariangela Rinaldi2, Jaime De Inocencio2, Erkan Demirkaya2, Stella Garay2, Dirk Foell2, Daniel J. Lovell2, Calin Lazar2, Susan Nielsen2, Berit Flato2, Alberto Martini2, Angelo Ravelli1, and EPOCA Study Group
1Pediatria II, Istituto Giannina Gaslini, Genova, Italy; 2PRINTO, Istituto Giannina Gaslini, Genova, Italy
Presenting author: Alessandro Consolaro
Africa | Asia | Eastern Europe | Latin America | North America | Western Europe | |
---|---|---|---|---|---|---|
N = 261 | N = 874 | N = 2587 | N = 814 | N = 422 | N = 3367 | |
Median (IQR) age at onset, years | 7 (3.6–10.5) | 5.7 (2.9–9.2) | 6.4 (2.9–10.5) | 6.6 (3.6–10.3) | 8.1 (3.7–11.1) | 4.1 (2–8.7) |
Systemic arthritis | 17.2 | 26.5 | 8.1 | 17.7 | 4.7 | 6.9 |
Oligoarthritis | 23 | 31.9 | 44.1 | 31.7 | 33.9 | 49.2 |
Uveitis | 4.6 | 5.5 | 10 | 6.6 | 10.4 | 16.9 |
Use of biologics | 22.6 | 23.6 | 28.9 | 34.2 | 47.6 | 39 |
Median (IQR) cJADAS10 | 7 (2–11) | 2.5 (0–7.8) | 4.5 (1–9.5) | 2.5 (0–9.5) | 2.3 (0–7) | 2 (0–6) |
Median (IQR) JAFS score | 4 (0–8) | 1 (0–5) | 1 (0–4) | 1 (0–5) | 1 (0–4) | 0 (0–3) |
JADI-Articular > 0 | 27.6 | 19.2 | 23.7 | 31.9 | 15.2 | 12.2 |
JADI-Extraarticular > 0 | 24.5 | 16.8 | 14.8 | 14.5 | 5.9 | 9.1 |
P64 Changes in switched memory B cells are associated to the therapeutic response to TNF inhibition in JIA
Emiliano Marasco1, Angela Aquilani1, Simona Cascioli2, Ivan Caiello1, Gian Marco Moneta1, Denise Pires-Marafón1, Silvia Magni-Manzoni1, Rita Carsetti2, Fabrizio De Benedetti1
1Division of Rheumatology, Ospedale Pediatrico Bambin Gesù, Roma, Italy; 2Immunology Diagnostic Unit, Ospedale Pediatrico Bambin Gesù, Roma, Italy
Presenting author: Emiliano Marasco
MTX with active disease (n = 4) | MTX with clinical remission (n = 10) |
t-Test (p-value) | MTX + TNFi with active disease (n = 19) | MTX + TNFi with clinical remission (n = 14) |
t-Test (p-value) | |
---|---|---|---|---|---|---|
% change from baseline of switched memory B cells (±SD) | 42%(±35) | 29.9%(±34) | 0.55 | 41%(±32) | 9.8%(±25) | 0.0047 |
Slope (indicating the mean % change of switched memory B cells in 12 months) (±SD) | 0.43(±0.35) | 0.42(±0.38) | 0.95 | 0.39(±0.32) | 0.09(±0.34) | 0.015 |
P65 Towards stratified medicine in juvenile idiopathic arthritis
Emily Robinson1, Salvatore Albani2, Michael W. Beresford3, Wilco de Jager4, Sytze de Roock4, Trang Duong5, Justine Ellis6, Kimme Hyrich7, Laetitia Jervis1, Daniel Lovell8, Lucy Marshall1, Elizabeth D. Mellins9, Kirsten Minden10, Jane Munro6, Peter A. Nigrovic11, Jason Palman1, Johannes Roth12, Nicolino Ruperto13, Sunil Sampath14, Laura E. Schanberg15, Susan D. Thompson8, Wendy Thomson14, Richard Vesely16, Chris Wallace17, Chris Williams18, Qiong Wu1, Nico Wulffraat4, Rae S. M. Yeung5, Berent Prakken4, Lucy R. Wedderburn1
1Institute of Child Health, University College London, London, United Kingdom; 2SingHealth Translational Immunology and Inflammation Centre, Singhealth and Duke-NUS Graduate Medical School, Singapore, Singapore; 3Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom; 4Department of Paediatric Immunology, University Medical Centre Utrecht, Utrecht, Netherlands; 5Division of Rheumatology, Hospital for Sick Children, University of Toronto, Toronto, Canada; 6Murdoch Children’s Research Institute, Royal Children’s Hospital, Melbourne, Australia; 7Arthritis Research UK Centre for Epidemiology, Centre for Musculoskeletal Research, University of Manchester, Manchester, United Kingdom; 8Division of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA; 9Department of Pediatrics, Standford University School of Medicine, Stanford, CA, USA; 10Paediatric Rheumatology, German Rheumatism Research Centre, Berlin, Germany; 11Division of Immunology, Boston Children’s Hospital, Boston, MA, USA; 12Institute of Immunology, University of Munster, Munster, Germany; 13Pediatria II Reumatologia, Instituto Giannina Gaslini, Genova, Italy; 14Arthritis Research UK Centre for Genetics and Genomics, Centre for Musculoskeletal Research, University of Manchester, Manchester, United Kingdom; 15Department of Pediatrics, Duke University Medical Center, Durham, NC, USA; 16Rheumatology, Immunology, Respiratory and Gastroenterology office, European Medicines Agency, London, UK; 17Department of Medicine, University of Cambridge and MRC Biostatistics unit, Cambridge, UK; 18UCL Business PLC, University College London, London, United Kingdom
Presenting author: Emily Robinson
P66 Safety of adalimumab in pediatric patients with polyarticular juvenile idiopathic arthritis, enthesitis-related arthritis, psoriasis, and Crohn’s disease
G Horneff1, MB Seyger2, D Arikan3, J Kalabic4, JK Anderson3, A Lazar4, DA Williams3, C Wang3, R Tarzynski-Potempa3, JS Hymans5
1Asklepios Clinic, Sankt Augustin, Germany; 2Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; 3AbbVie Inc., North Chicago, IL, USA; 4AbbVie Deutschland GmbH & Co. KG, Ludwigshafen, Germany; 5Connecticut Children’s Medical Center, Hartford, CT, USA
Presenting author: G Horneff
P67 Flares after withdrawal of biotherapies in JIA: clinical and laboratory correlates of remission duration
Gabriele Simonini1, Erika Scoccimarro1, Irene Pontikaki2, Giovanna Ferrara3, Teresa Giani1, Alessandro Ventura3, Pier Luigi Meroni4, Rolando Cimaz1
1Rheumatology Unit-Dpt of Paediatrics University of Florence-Anna Meyer Children Hospital, Firenze, Italy; 2Gaetano Pini Institute, Milano, Italy; 3Institute for Maternal and Child Health - IRCCS “Burlo Garofolo”, University of Trieste, Trieste, Italy; 4Gaetano Pini Institute, University of Milano, Milano, Italy
Presenting author: Gabriele Simonini
P68 An active proNGF-p75NTR axis plays a pro-inflammatory role in chronic arthritis
Gaetana Minnone1, Marzia Soligo2, Ivan Caiello1, Giusi Prencipe1, Denise Pires Marafon1, Silvia Magni-Manzoni1, Luigi Manni2, Fabrizio De Benedetti1, Luisa Bracci Laudiero2
1Division of Rheumatology, Bambino Gesu’ Children’s Hospital, Rome, Italy; 2Institute of Translational Pharmacology, Consiglio Nazionale delle Ricerche (CNR), Rome, Italy
Presenting author: Gaetana Minnone
P69 Humoral and cellular responses to varicella vaccination of patients with pediatric rheumatic diseases – preliminary results of a vaccination study
Noortje Groot1,2, I Grein2,3, N.M. Wulffraat2, R Schepp4, G Berbers4, C. C. Barbosa Sandoval de Souza5, V. Paes Leme Ferriani5, G Pileggi5, S. de Roock6
1Department of Pediatric Rheumatology, Sophia Children’s Hospital, Erasmus University Medical Centre, Rotterdam, Netherlands; 2Department of Pediatric Immunology and Rheumatology, Wilhelmina Children’s Hospital, University Medical Centre, Utrecht, Netherlands; 3Department of Pediatric Rheumatology, Pequeno Príncipe Hospital, Curitiba, Brazil; 4National Institute of Public Health and the Environment (RIVM), Bilthoven, Netherlands; 5Department of Pediatrics, University of Medicine of Ribeirão Preto, São Paulo, Brazil; 6Wilhelmina Children’s Hospital, University Medical Centre, Utrecht, Netherlands
Presenting author: I Grein
Patients (n = 54) | Healthy controls (n = 18) | |
---|---|---|
Disease type | N/A | |
Systemic JIA | 11 (20%) | |
Oligo JIA | 9 (17%) | |
Poly JIA | 22 (41%) | |
JScl | 5 (9%) | |
JDM | 7 (13%) | |
Age at vaccination (median, range)
| 5 (0–20) | 8.5 (3–18) |
Medication use | N/A | |
MTX | 98% | |
MTX dose (median, range)
| 15 (10–27) | |
Corticosteroids | 43% | |
Steroid dose (median, range)
| 6 (3–20) | |
Biologics | 6% | |
Responder | 17 | 6 |
Nonresponder | 18 | 12 |
P70 Share update – 508 families view on standard of care for children with Juvenile Idiopathic Arthritis in Europe and Israel
Ingrid H. R. Grein1,2, Silvia Scala3, Elisa Patrone3, Casper Schoemaker4, on behalf of Dutch JIA patient organization, Wendy Costello5, on behalf of ENCA, Nico Wulffraat2
1Pediatric Rheumatology Department, Hospital Pequeno Príncipe, Curitiba, Brazil; 2Pediatric Immunology and Rheumatology Department, Wilhemina Children’s Hospital/University Medical Centre Utrecht, Utrecht, Netherlands; 3Research Department, Istituto Giannina Gaslini, Genoa, Italy; 4Dutch JIA patient organization, Wilhemina Children’s Hospital/University Medical Centre Utrecht, Utrecht, Netherlands; 5Chair of European Network for Children with Arthritis (ENCA), Tipperary, Ireland
Presenting author: Ingrid H. R. Grein
West-Europe | East-Europe | Asia | |||||
---|---|---|---|---|---|---|---|
Netherlands | 100 | Germany | 28 | Latvia | 21 | Israel | 29 |
Belgium | 16 | Austria | 1 | Lithuania | 2 | ||
Denmark | 34 | Switzerland | 1 | Czech Republic | 20 | ||
Sweden | 1 | Italy | 26 | Serbia | 5 | ||
United Kingdom | 33 | Portugal | 5 | Slovakia | 33 | ||
Ireland | 26 | Spain | 20 | Slovenia | 17 | ||
France | 59 | Albania | 1 | ||||
Greece | 30 | ||||||
TOTAL = 350 | TOTAL = 129 | TOTAL = 29 |
P71 Young people’s beliefs about research priorities in rheumatology research
Suzanne Parsons1, Janet McDonagh2, Wendy Thomson2
1Public Programmes Team, Central Manchester University Hospitals’ NHS Foundation Trust, Manchester, UK; 2Centre for Musculoskeletal Research, University of Manchester, Manchester, United Kingdom
Presenting author: Suzanne Parsons
P72 Development of the first video game aimed at children suffering from Juvenile Idiopathic Arthritis
Jean-David Cohen1, Damien Bentayou2, Marc-Antoine Bernard Brunel2, Sonia Trope3
1Public Hospital, Montpellier, France; 2CG Artists, Bordeaux, France; 3ANDAR patient organization, Paris, France
Presenting author: Jean-David Cohen
P73 JIA patients have a similar quality of life as healthy peers after three years of specialised care
Jens Klotsche1, Miriam Listing1, Martina Niewerth1, Gerd Horneff2, Angelika Thon3, Hans-Iko Huppertz4, Kirsten Mönkemöller5, Ivan Foeldvari6, Dirk Föll7, Kirsten Minden1, and ICON study group
1Epidemiology unit, German Rheumatism Research Centre, Berlin, Germany; 2Kinderklinik, Asklepios, Sankt Augustin, Germany; 3Kinderklinik, Medizinischen Hochschule Hannover, Hannover, Germany; 4Prof.-Hess-Kinderklinik, Klinikum Bremen-Mitte, Bremen, Germany; 5Klinik für Kinder- und Jugendmedizin, Kinderkrankenhauses der Stadt Köln, Köln, Germany; 6Hamburger Zentrum für Kinder- und Jugendrheumatologie, am Klinikum Eilbek, Hamburg, Germany; 7Klinik für pädiatrische Rheumatologie und Immunologie, Universitätsklinikum Münster, Münster, Germany
Presenting author: Kirsten Minden
P74 Auxological features in patients with Juvenile Idiopathic Arthritis (JIA) treated with biologic drugs
Achille Marino1, Stefano Stagi2, Niccolò Carli1, Federico Bertini1, Teresa Giani1, Gabriele Simonini1, Rolando Cimaz1
1Department of Paediatrics, Rheumatology Unit, Anna Meyer Children’s Hospital, University of Florence, Florence, Italy; 2Department of Paediatrics, Endocrinology Unit, Anna Meyer Children’s Hospital, University of Florence, Florence, Italy
Presenting author: Achille Marino
P75 Response to conventional DMARD treatment in a cohort of children with Juvenile Idiopathic Arthritis in Bogota, Colombia
Adriana S. Díaz-Maldonado1, Sally Pino2, Pilar Guarnizo3
1Pediatric Rheumatology, Care for Kids - Hospital La Misericordia - Instituto Roosevelt, Bogotá, Colombia; 2Pediatric Rheumatology, Care for Kids - Hospital Infantil de San José, Bogotá, Colombia; 3Pediatric Rheumatology, Care for Kids - Fundación Cardio Infantil - CAYRE, Bogotá, Colombia
Presenting author: Adriana S. Díaz-Maldonado
P76 Discrimination between Juvenile Idiopathic Arthritis and leukemic arthritis
Alfonso Ragnar Torres-Jimenez1, Berenice Sanchez-Jara2, Eunice Solis-Vallejo1, Adriana Ivonne Cespedes-Cruz1, Maritza Zeferino-Cruz1, Julia Veronica Ramirez-Miramontes1
1Reumatologia Pediatrica, Imss Hospital General Centro Medico Nacional La Raza, Azcapotzalco, Mexico; 2Hematologia Pediatrica, Imss Hospital General Centro Medico Nacional La Raza, Azcapotzalco, Mexico
Leukemic arthritis (n = 9) | JIA (N = 25) |
P value | RR (IC 95%) | |
---|---|---|---|---|
Pain intensity (0–10) | 10 | 6.6 (4–10) | *0.0001 | |
Night pain | 66% | 4% | *0.0001 | 7.7 (2.5–23) |
Fever | 66% | 12% | *0.001 | 5 (1.7–17.6) |
Weight loss | 78% | 16% | *0.001 | 7.3 (1.8–29.6) |
Lymphadenopathy | 88% | 4% | *0.0001 | 22 (3.2–153.7) |
Anemia Hemoglobin g/dL | 56% | 8% | *0.002 | 4.8 (1.7–13.3) |
10.9 (8.3–12.9) | 13.1 (10.2–16.4) | |||
Leukopenia Leukocytes mm3
| 67% | 0% | *0.0001 | 9.3 (3–27) |
3555 (1610–5530) | 9750 (5190–24630) | |||
Neutropenia Neutrophil mm3
| 88% | 0% | *0.0001 | 25 (3.3–170) |
473 (0–1820) | 6456 (2270–22130) | |||
Thrombocytopenia Platelets ×103/mm3
| 44% | 0% | *0.0001 | 6 (2.6–13) |
171 (53–296) | 375 (207–661) |
P77 Rheumatoid factor positive polyarticular JIA with pulmonary langerhans cell histiocytosis- a chance association or a causal relationship?
Ankur Kumar1, Anju Gupta1, Deepti Suri1, Amit Rawat1, Nandita Kakkar2, Surjit Singh1
1Pediatrics, PGIMER, Chandigarh, India; 2Histopathology, PGIMER, Chandigarh, India
Presenting author: Ankur Kumar
Investigation | Result |
---|---|
Haemoglobin (gm/L) | 119 |
WBC counts (×109 cells/L) | 11 |
Differential counts | N63/L23/M7/E7
|
Platelet counts (×109 /L) | 467 |
ESR (mm/hr) | 49 |
CRP (mg/dL) | 18 |
AST (U/L) | 49 |
ALT (U/L) | 30 |
ALP (U/L) | 190 |
Anti nuclear antibody | 3+ homogenous |
Rheumatoid factor (U/ml) | |
At diagnosis | 181 (normal range: 0–19) |
4 months later | 199 |
P78 The rate of obesity and overweight among Turkish children with Juvenile Idiopathic Arthritis
Balahan Makay, Özge A. Gücenmez, Erbil Ünsal
Pediatric Rheumatology, Dokuz Eylül University Hospital, İzmir, Turkey
Presenting author: Balahan Makay
Healthy weight | Increased weight | |
---|---|---|
Persistent oligoarthritis | 95 (90.5) | 10 (9.5) |
ERA | 55 (68) | 26 (32) |
Poliarthritis RF (+) | 46 (78) | 13 (22) |
Systemic | 28 (90.5) | 1 (9.5) |
Undifferentiated | 17 (81) | 4 (19) |
Psoriatic | 16 (100) | 0 (0) |
Extended oligoarthritis | 8 (89) | 1 (11) |
Poliarthritis RF (-) | 4 (80) | 1 (20) |
P79 Patient partnership and disabkids in the Swedish Pediatric Rheumatology Registry
Bo Magnusson1, Karina Mördrup1, Anna Vermé1, Christina Peterson2, and Board of the Swedish Pediatric Rheumatology Registry
1Paediatric Rheumatology Unit, Astrid Lindgren Children’s Hospital, Karolinska University hospital, Stockholm, Sweden; 2Hälsohögskolan, Jönköping, Sweden
Presenting author: Bo Magnusson
Independence | Innerstrength | Social Inclusion | Social Equality | Physical ability | Physical Treatment | Total score | Impact | Understanding |
---|---|---|---|---|---|---|---|---|
76% | 47% | 67% | 67% | 65% | 64% | 58% | 2.7/5 | 2.6/5 |
P80 Diagnostic duration and access to care in JIA: a pilot study in the University Hospital of Saint Etienne (France)
Caroline Freychet1, Jean Louis Stephan2, Michael Hofer3, Alexandre Belot4
1Pediatrics, University Hospital of St Etienne, St Etienne, France; 2Pediatrics, University Hospital of St Etienne, Saint Etienne, France; 3Pediatrics, University Hospital of Lausanne, Lausanne, Switzerland; 4Pediatrics, University Hospital of Lyon, Lyon, France
Presenting author: Caroline Freychet
P81 Effectiveness of knee splintage under general anaesthetic post interarticular injection in children suffering from JIA
Cathryn E. Harkness1, Madeleine Rooney2, Leanne Foster1, Emma Henry1, Pauline Taggart2
1Occupational Therapy Musgrave Park Hospital, Belfast, United Kingdom, 2Belfast Health & Social Care Trust, Belfast, United Kingdom
Presenting author: Cathryn E. Harkness
Jt 1 | 90% |
Jt 2 | 80% |
Jt 3 | 92% |
Jt 4 | 67% |
Jt 5 | 90% |
Jt 6 | 67% |
Jt 7 | 80% |
Jt 8 | 100% |
Jt 9 | 63% |
Jt 10 | 100% |
P82 Analysis of disease activity and disease damage in patients with Juvenile Idiopathic Arthritis: single center experience
Dogan Simsek1, Coskun F. Ozkececi2, Esra Kurt1, Gokalp Basbozkurt2, Faysal Gok1, Erkan Demirkaya1
1Pediatric Rheumatology, Gulhane Military Medical Academy, Ankara, Turkey; 2Pediatrics, Gulhane Military Medical Academy, Ankara, Turkey
Presenting author: Dogan Simsek
P83 Serum levels of myeloid-related proteins 8 and 14 in oligoarticular and polyarticular onset of Juvenile Idiopathic Arthritis
Daiva Gorczyca1, Jacek Postępski2, Aleksandra Czajkowska3, Bogumiła Szponar3, Mariola Paściak3, Anna Gruenpeter4, Iwona Lachór-Motyka4, Daria Augustyniak5, Edyta Olesińska6
1Third Department and Clinic of Paediatrics, Immunology and Rheumatology of Developmental Age, Wroclav Medical University, Wroclaw, Poland; 2Department of Paediatric Pulmonology and Rheumatology, Medical University of Lublin, Lublin, Poland; 3Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Wroclaw, Poland; 4Department of Paediatric Rheumatology, John Paul II Paediatric Centre, Sosnowiec, Poland; 5Department of Pathogen Biology and Immunology, Institute of Genetics and Microbiology, Wroclav University, Wroclaw, Poland; 6Department of Paediatric Pulmonology and Rheumatology, Children’s University Hospital, Lublin, Poland
Presenting author: Edyta Olesińska
P84 Juvenile Idiopathic Arthritis in mono-zygotic twin
Emediong S. Asuka1, Tatyana Golovko1, Samuel U. Aliejim1
1Pediatrics Department, Vasyl Karazin Kharkiv National University, Kharkiv, Ukraine
Presenting author: Emediong S. Asuka
P85 Diagnostic pitfalls in Juvenile Idiopathic Arthritis: beyond inflammation
Emilio Inarejos Clemente1, Estibaliz Iglesias Jimenez2, Joan Calzada Hernandez2, Sergi Borlan Fernandez2, Clara Gimenez Roca2, David Moreno Romo3, Natalia Rodriguez Nieva4, Juan Manuel Mosquera Angarita2, Jordi Anton Lopez2
1Radiology, Hospital Sant Joan de Deu, Barcelona, Spain; 2Rheumatology, Hospital Sant Joan de Deu, Barcelona, Spain; 3Orthopedics, Hospital Sant Joan de Deu, Barcelona, Spain; 4Rehabilitation, Hospital Sant Joan de Deu, Barcelona, Spain
Presenting author: Emilio Inarejos Clemente
P86 Insulin resitance and other cardiovascular disease risk factors in Juvenile Idiophatic Arthritis
Esmeralda Nuñez-Cuadros1, Gisela Diaz-Cordovés1, Rocío Galindo-Zavala1, Antonio Urda-Cardona2, Antonio Fernández-Nebro3
1Pediatric Rheumatology Unit, Regional Universitary Hospital of Málaga, Málaga, Spain; 2Pediatric Department, H. Materno-Infantil. HRU de Málaga, Málaga, Spain; 3Rheumatology Department, Regional Universitary Hospital of Málaga, Málaga, Spain
Presenting author: Esmeralda Nuñez-Cuadros
N = 88 | |
---|---|
JIA subtype, n (%) | |
Systemic 10 (11,4) | |
Persistent oligoarticular 43 (48.9) | |
Extended oligoarticular 14 (15.9) | |
Polyarticular 21 (23.8) | |
Disease duration (years), mean ± SD | 6,3 ± 3,6 |
Inflammatory activity duration (weeks), median (IR) | 53 (32–109) |
Synthetic DMARDs treatment, n (%) | 85 (96.5) |
Biological DMARDs treatment, n (%) | 40 (45.5) |
Systemic corticosteroids, n (%) | 79 (89.7) |
Duration corticosteroids (days), median (IR) | 122 (55–178) |
Median corticosteroids dose (mg/kg/day), median (IR) | 0.34 (0.21–1.15) |
P87 Prospective analysis of the immunogenic response in JIA patients (paediatric and adult) on antitnf treatment
Estefania Quesada-Masachs1, Daniel Álvarez de la Sierra2, Marina Garcia Prat2, Mónica Martínez Gallo2, Ricardo Pujol Borrell2, Sara Marsal Barril3, Ana M. Marín Sánchez2, Consuelo Modesto Caballero1
1Paediatric Rheumatology Unit, Vall d’Hebron University Hospital, Barcelona, Spain; 2Immunology, Vall d’Hebron University Hospital, Barcelona, Spain; 3Rheumatology, Vall d’Hebron University Hospital, Barcelona, Spain
Presenting author: Estefania Quesada-Masachs
P88 Fibrous arthropathy associated with morphea: a new cause of diffuse acquired joint contractures in children
Etienne Merlin1, Sylvain Breton2, Sylvie Fraitag2, Jean-Louis Stephan3, Carine Wouters4, Christine Bodemer2, Brigitte Bader-Meunier2
1CHU Clermont-Ferrand, Clermont-Ferrand, France; 2APHP, Paris, France; 3CHU Saint-Etienne, Saint-Priest en Jarrez, France; 4University Hospital Leuven, Leuven, Belgium
Presenting author: Etienne Merlin
P89 Subclinical atherosclerosis in children with Juvenile Idiopathic Arthritis
Francesco Baldo1, Federico Annoni2, Giancarla Di Landro1, Sofia Torreggiani1, Marta Torcoletti1, Antonella Petaccia1, Fabrizia Corona1, Giovanni Filocamo1
1Dipartimento della Donna, del Bambino o del Neonato, Fondazione Irccs Cà Granda Ospedale Maggiore Policlinico, Milano, Italy; 2Dipartimento di Fisiopatologia Medico Chirurgica e dei Trapianti, Fondazione Irccs Cà Granda Ospedale Maggiore Policlinico and Università di Milano, Milano, Italy
Presenting author: Francesco Baldo
IMT | |
---|---|
Age | 0,518340 |
Age at onset | 0,380243 |
Disease duration | 0,303138 |
Physician’s global assessment | -0,066940 |
Active joint count | 0,013623 |
ESR | -0,107416 |
Cholesterol level | 0,589938 |
Weight | 0,562500 |
Blood pressure | -0,217043 |
P90 Treatment and 1-year outcomes of an inception cohort of Australian children with JIA
Georgina Tiller, Jo Buckle, Jane Munro, Angela Cox, Peter Gowdie, Roger C. Allen, Jonathan D. Akikusa
Rheumatology, Royal Childrens Hospital, Melbourne, Australia
Presenting author: Georgina Tiller
P91 Anti-cyclic citrullinated peptide antibodies, antinuclear antibodies, and rheumatoid factor in Mexican patients with Juvenile Idiopathic Arthritis
Hayde G. Hernández-Huirache1, Edel R. Rodea-Montero2
1Pediatric Rheumatology, Hospital Regional de Alta Especialidad del Bajío, Leon, Guanajuato, Mexico; 2Research, Hospital Regional de Alta Especialidad del Bajío, Leon, Guanajuato, Mexico
Presenting author: Hayde G. Hernández-Huirache
Variables | Systemic arthritis (n = 8) | Enthesitis related to arthritis (n = 4) | Oligoarthritis (n = 7) | Seronegative polyarthritis (n = 14) | Seropositive polyarthritis (n = 17) | p-value |
---|---|---|---|---|---|---|
Female, n (%) | 2 (25%) | 0 (0%) | 4 (57%) | 10 (71%) | 13 (76%) | p = 0.014a,c
|
Male, n (%) | 6 (75%) | 4 (100%) | 3 (43%) | 4 (29%) | 4 (24%) | p = 0.014a,c
|
Age (years) | 8.76 (4.86) | 13.70 (1.91) | 7.66 (3.49) | 9.31 (4.21) | 12.9 (1.98) | p = 0.007b,c
|
RF (UI/ml) | 8.28 (0.92) | 8.60 (0.00) | 8.47 (1.09) | 8.6 (0.00) | 196.82 (300.65) | p < 0.001b,c
|
Anti-CCPA (U/ml) | 9.41 (9.44) | 11.95 (20.45) | 6.29 (7.36) | 9.11 (14.17) | 64.94 (84.49) | p < 0.001b,c
|
<20 (negative) n (%) | 6 (75%) | 3 (75%) | 6 (86%) | 11 (79%) | 3 (18%) | p < 0.001a,c
|
≥20 (positive) n (%) | 2 (25%) | 1 (25%) | 1 (14%) | 3 (21%) | 14 (82%) | p < 0.001a,c
|
P92 Development of the “REAJI” skill framework in Juvenile Idiopathic Arthritis
Jean-David Cohen1, Alexandre Belot2, William Fahy3, Pierre Quartier4, Christelle Sordet5, Sonia Trope6
1Public Hospital, Montpellier, France; 2Public Hospital, Lyon, France; 3KOURIR patient organization, Paris, France; 4Public Hospital Necker, Paris, France; 5Public Hospital, Strasbourg, France; 6ANDAR patient organization, Paris, France
Presenting author: Jean-David Cohen
P93 Measuring grip strength in children and adolescents diagnosed with juvenile idiopathic arthritis (JIA) using the new computerised Grippit™. Does poor grip strength and pain affect activities in daily life?
Karin B. Berggren, Johanna T. Kembe
Occupational Therapy Clinic, Children’s Occupational Therapy Ward, Stockholm, Sweden
Presenting author: Johanna T. Kembe
P94 Internet program for physical activity and exercise-capacity in children with Juvenile Idiopathic Arthritis; a multicenter randomized controlled trial
Joyce Bos1, Wineke Armbrust2, Nico Wulffraat3, Marco van Brussel4, Jeanette Cappon5, Pieter Dijkstra6, Jan Geertzen1, Elizabeth Legger2, Marion van Rossum5, Pieter Sauer7, Otto Lelieveld1
1Center for Rehabilitation, UMCG, Groningen, Netherlands; 2Department of Pediatric Rheumatology, Beatrix Children’s Hospital, UMCG, Groningen, Netherlands; 3Department of Pediatric Immunology, Wilhelmina Children’s Hospital, UMCU, Groningen, Netherlands; 4Child Development & Exercise Center, Wilhelmina Children’s Hospital, UMCU, Utrecht, Netherlands; 5Center for Rehabilitation and Rheumatology, Reade, Amsterdam, Netherlands; 6Center for Rehabilitation and Dept. of Oral and Maxillofacial Surgery, UMCG, Utrecht, Netherlands; 7Beatrix Children’s Hospital, UMCG, Groningen, Netherlands
Presenting author: Joyce Bos
P95 A rare disease in the elbow: Lipoma Arborescens
Kubra Ozturk1, Levent Buluc2, Gur Akansel3, Bahar Muezzinoglu4, Zelal Ekinci1
1Department of Pediatrics Rheumatology, Kocaeli University Faculty of Medicine, Kocaeli, Turkey; 2Department of Orthopaedics and Traumatology, Kocaeli University Faculty of Medicine, Kocaeli, Turkey; 3Department of Radiology, Kocaeli University Faculty of Medicine, Kocaeli, Turkey; 4Department of Pathology, Kocaeli University Faculty of Medicine, Kocaeli, Turkey
Presenting author: Kubra Ozturk
P96 Risk factors of bone mineral density decrease in children with juvenile arthritis
Ljubov Rychkova, Tatyana Knyazeva, Anna Pogodina, Tatyana Belova, Tamara Mandzyak, Ekaterina Kulesh
Scientific Center Of The Family Health And Human Reproduction Problems, Irkutsk, Russian Federation
Presenting author: Ljubov Rychkova
P97 Renal function in children with Juvenile Idiopathic Arthritis: a case-control study
Alessandro Cafarotti1, Manuela Marsili1, Cosimo Giannini1, Roberta Salvatore1, Giuseppe Lapergola1, Caterina Di Battista1, Maria Loredana Marcovecchio1, Raffaella Basilico2, Piernicola Pelliccia1, Francesco Chiarelli1, Luciana Breda1
1Department of Pediatrics, University of Chieti, Chieti, Italy; 2Department of Radiology, University of Chieti, Chieti, Italy
Presenting author: Manuela Marsili
Parameters | Controls (n = 51) | JIA MTX group (n = 20) | JIA BD group (n = 26) | p-value*†‡§ |
---|---|---|---|---|
Creatinine (mg/dl) | 0.42 ± 0.10 | 0.48 ± 0.08 | 0.54 ± 0.15 | <0.001†‡
|
Cystatin C (mg/l) | 0.66 ± 0.05 | 0.79 ± 0.06 | 0.77 ± 0.13 | <0.001†‡
|
BUN (mg/dl) | 11.72 ± 1.32 | 12.00 ± 2.59 | 13.61 ± 3.18 | 0.002‡
|
Microalbuminuria (mg/dl) | 1.09 ± 0.00 | 1.29 ± 0.60 | 1.54 ± 1.55 | 0.08 |
ESR (mm/h) | 1.00 ± 0.00 | 9.15 ± 6.64 | 15.00 ± 8.68 | <0.001†‡§
|
CRP (mg/dl) | 0.20 ± 0.00 | 0.34 ± 0.31 | 0.78 ± 084 | <0.001‡§
|
eGFR (ml/min/1.73 m2) | 121.9 ± 19.6 | 98.9 ± 8.3 | 98.1 ± 10.7 | <0.001†‡
|
RI | 0.60 ± 0.03 | 0.65 ± 0.02 | 0.68 ± 0.03 | <0.001†‡§ |
Duration of disease (years) | - | 3.96 ± 2.72 | 6.59 ± 3.18 | 0.35 |
Poster Session: Juvenile dermatomyositis I
P98 Anti-synthetase autoantibody is seen in patients with overlap myositis in the UK cohort of patients with jveunile dermatomyositis
Beverley Almeida1, Sarah Tansley2, Stefania Simou1, Harsha Gunawardena3, Neil McHugh4, Lucy Wedderburn1, and Juvenile Dermatomyositis Research Group (JDRG)
1Rheumatology, Institute of Child Health, University College London, London, UK; 2Rheumatology, Royal National Hospital For Rheumatic Diseases, Bath, UK; 3Clinical and Academic Rheumatology, North Bristol NHS Trust, Southmead Hospital, Bristol, UK; 4Department of Pharmacy and Pharmacology, University of Bath, Bath, United Kingdom
Presenting author: Beverley Almeida
Patient | 1 | 2 | 3 | 4 | 5 | 6 |
---|---|---|---|---|---|---|
Diagnostic label | Overlap with scleroderma and arthritis | Overlap with scleroderma and arthritis | Overlap with arthritis | Initially diagnosed with PAN, then myositis and SLE | JDM | Overlap with systemic sclerosis |
Age at onset (years) | 9.06 | 9.41 | 15.47 | 13.06 | 12.53 | 14.43 |
Length of follow up (years) | 0.67 | 11.5 | 1.33 | 6.47 | 3.5 | 2.76 |
Specific antibody | Jo1 | Jo1 | Jo1 | PL12 | PL7 | PL12 |
Muscle | Severe myositis | Myositis | Myositis | Myositis | Myositis | Destructive necrotizing, myopathy on biopsy |
Skin | Gottron’s Heliotrope rash | Gottron’s Mechanics hands | Gottron’s Heliotrope rash | Heliotrope rash Erythematous rash on forearms | Gottron’s Heliotrope rash | Gottron’s Heliotrope rash Skin ulceration and calcinosis Mechanics hand |
Joint | Polyarthritis with contractures | Nil of note | Arthritis | Nil of note | Nil of note | Nil of note |
Lung | Normal | Normal | Normal | ILD | ILD | ILD |
Mortality to date | Alive | Alive | Alive | Alive | Alive | Alive |
P99 Positive detection of Anti-NXP2 autoantibodies correlates with muscle ischemia in juvenile dermatomyositis
Jessie Aouizerate1, Brigitte Bader-Meunier2, Marie De Antonio1, Christine Bodemer2, Christine Barnerias2, Guillaume Bassez1, Isabelle Desguerre2, Pierre Quartier2, Romain Gherardi2, Jean-Luc Charuel3, François-Jérôme Authier1, Cyril Gitiaux2
1Hôptal H.Mondor, Creteil, Paris, France, 2Hôpital Necker, Paris, France, 3Hôpital La Pitié, Paris, France
Presenting author: Brigitte Bader-Meunier
P100 The role of muscle MRI in detecting a flare-up of juvenile dermatomyositis
C H. Spencer1, Rabheh Abdul Aziz1, Chack-Yung Yu1, Brent Adler2, Sharon Bout-Tabaku1, Katherine Lintner3, Melissa Moore-Clingenpeel3
1Rheumatology, Nationwide Childrens/Ohio State, Columbus, United States; 2Radiology, Nationwide Childrens/Ohio State, Columbus, United States; 3Research Institute, Nationwide Childrens/Ohio State, Columbus, United States
Presenting author: C H. Spencer
P101 Juvenile myositis: what happens next?
Christina Boros1,2, Liza McCann3, Nicola Ambrose4, Mario Cortina-Borja2, Stefania Simou2, Clarissa Pilkington5, Lucy Wedderburn2, and Juvenile Dermatomyositis Cohort and Biomarker Study (JCDBS)
1University of Adelaide, Adelaide, Australia; 2University College London Institute of Child Health, London, UK; 3Alder Hey Childrens Hospital, Liverpool, UK; 4University College Hospital London, UK; 5Great Ormond Street Hospital for Children, London, United Kingdom
Category | Patient reported outcome |
n (%) answering yes |
---|---|---|
Specific disease features | Muscle weakness | 28 (32.5) |
Arthritis | 28 (32.5) | |
Skin rash | 21 (25.3) | |
Calcinosis | 18 (21.9) | |
Education and Employment status | At school | 17 (20.5) |
Enrolled in higher education | 15 (18.1) | |
Employed | 38 (45.8) | |
Unemployed | 14 (16.9) |
P102 Diagnosis of Juvenile Dermatomyositis in Germany – a survey among paediatric rheumatologists and child neurologists
Claas Hinze1, Prasad T. Oommen2, Fabian Speth3, Johannes-Peter Haas3, and Working Group “Juvenile Dermatomyositis” of the German Society for Paediatric and Adolescent Rheumatology (GKJR)
1Pediatric Rheumatology and Immunology, University Hospital Münster, Münster, Germany; 2Department of Pediatric Oncology, Hematology and Immunology, University Children’s Hospital, Medical Faculty, Heinrich-Heine-University, Düsseldorf, Germany; 3German Center for Paediatric Rheumatology and Immunology, Garmisch-Partenkirchen, Germany
Presenting author: Claas Hinze
P103 Treatment of Juvenile Dermatomyositis in Germany – a survey among pediatric rheumatologists and child neurologists
Claas Hinze1, Prasad T. Oommen2, Fabian Speth3, Johannes-Peter Haas3, and Working Group “Juvenile Dermatomyositis” of the German Society for Paediatric and Adolescent Rheumatology (GKJR)
1Pediatric Rheumatology and Immunology, University Hospital Münster, Münster, Germany; 2Department of Pediatric Oncology, Hematology and Immunology, University Children’s Hospital, Medical Faculty, Heinrich-Heine-University, Düsseldorf, Germany; 3German Center for Paediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany
Presenting author: Claas Hinze
P104 Treatment with high-dose subcutaneous immune globulins facilitated by recombinant human hyaluronidase in patients with juvenile dermatomyositis who are intolerant to intravenous immune globulins
Fabian Speth1, Johannes-Peter Haas1, Claas Hinze2
1German Center for Paediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany; 2Pediatric Rheumatology and Immunology, University Hospital Münster, Münster, Germany
Presenting author: Claas Hinze
P105 Comparison of the PRINTO 2010 and PRINTO/IMACS 2016 improvement criteria in the PRINTO trial in new onset juvenile dermatomyositis
Claudio Lavarello, Gabriella Giancane, Angela Pistorio, Lisa Rider, Rohit Aggarwal, Sheila K. Oliveira, Ruben Cuttica, Michel Fischbach, Gary Sterba, Karine Brochard, Frank Dressler, Patrizia Barone, Ruben Burgos-Vargas, Elizabeth Candell Chalom, Marine Desjonqueres, Graciela Espada, Anders Fasth, Stella Maris Garay, Rose-Marie Herbigneaux, Claire Hoyoux, Chantal Job Deslandre, Frederick W. Miller, Jiri Vencovsky, Angelo Ravelli, Alberto Martini, Nicolino Ruperto
Pediatria II, Reumatologia; PRINTO, Istituto Giannina Gaslini, GENOA, Italy
Presenting author: Claudio Lavarello
2010 or 2016 criteria of improvement | PDN | PDN + CSA | PDN + MTX | P values |
---|---|---|---|---|
N = 47 | N = 46 | N = 46 | ||
N (%) [95%CI] | N (%) [95%CI] | N (%) [95%CI] | ||
Month 6 | ||||
2010 ≥ 20% | 24 (51%) [36–66] | 32 (70%) [54–82] | 33 (72%) [56–84] | 0.023 |
2016 at least minimal | 26 (55%) [40–70] | 34 (74%) [59–86] | 34 (74%) [59–86] | 0.027 |
Month 18 | ||||
2010 ≥ 50% | 20 (42%) [28–58] | 30 (65%) [50–79] | 29 (63%) [47–77] | 0.015 |
2016 at least moderate | 21 (45%) [30–60] | 30 (65%) [50–79] | 30 (65%) [50–79] | 0.020 |
Month 24 | ||||
2010 ≥ 70% | 18 (38%) [24–54] | 26 (56%) [41–71] | 26 (56%) [41–71] | 0.042 |
2016 at least major | 17 (36%) [23–51] | 27 (59%) [43–73] | 27 (59%) [43–73] | 0.012 |
P106 Inflammatory milieu of muscle biopsies and clinical features in juvenile dermatomyositis
Erdal Sag1, Seza Ozen2, Gulsev Kale1, Haluk Topaloglu3, Beril Talim1
1Department of Pediatrics, Hacettepe University, Faculty of Medicine, Ankara, Turkey; 2Division of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey; 3Division of Pediatric Neurology, Hacettepe University, Faculty of Medicine, Department of Pediatrics, Ankara, Turkey
Presenting author: Erdal Sag
P107 Evidence based criteria for corticosteroid tapering/discontinuation. An analysis of the PRINTO trial in new onset juvenile dermatomyositis
Gabriella Giancane, Claudio Lavarello, Angela Pistorio, Francesco Zulian, Bo Magnusson, Tadej Avcin, Fabrizia Corona, Valeria Gerloni, Serena Pastore, Roberto Marini, Silvana Martino, Anne Pagnier, Michel Rodiere, Christine Soler, Valda Stanevicha, Rebecca Ten Cate, Yosef Uziel, Jelena Vojinovic, Angelo Ravelli, Alberto Martini, Nicolino Ruperto
Pediatria II, Reumatologia; PRINTO, Istituto Giannina Gaslini, Genoa, Italy
Presenting author: Gabriella Giancane
OFF PDN | OFF PDN | PDN ON | P value | |
---|---|---|---|---|
Failure: No | Failure: Yes | Failure Yes or No | ||
Group 1 | Group 2 | Group 3 | ||
N = 57 | N = 24 | N = 58 | ||
Month 6: MD global | 0.5 (0–2) | 2.6 (0.8–5) | 3 (0.6–6) | <0.0001 |
Parent global | 1 (0–1.8) | 3.1 (0.5–5) | 2.5 (0.8–5.8) | 0.0002 |
CHAQ | 0.1 (0–0.5) | 0.1 (0–0.9) | 0.5 (0–1.9) | 0.0007 |
DAS | 3 (0–5) | 6.5 (1–11.5) | 8 (4–12) | <0.0001 |
CMAS | 47 (42–51) | 45 (35–48.5) | 35 (21.5–47) | 0.0001 |
CHQ-PhS | 51.8 (46.2–54.9) | 47.5 (20.3–54.1) | 36.3 (16.6–50.6) | <0.0001 |
MMT | 77 (70–80) | 70 (59–78.5) | 64 (51–75) | <0.0001 |
Month 12: MD global | 0 (0–1) | 2.6 (1–5.5) | 3 (0.6–6) | <0.0001 |
Month 18 DAS | 0 (0–3) | 6 (2.5–11.5) | 9 (2–12) | <0.0001 |
Month 24: CMAS | 50 (48–52) | 42 (36–50) | 36.5 (21.5–48) | <0.0001 |
P108 Juvenile dermatomyositis, predictors of disease progression in Mexican children
Ana V. Villarreal, Nydia Acevedo, Talia Diaz, Yuridiana Ramirez, Enrique Faugier, Rocio Maldonado
Pediatrics Rheumatology, Hospital Infantil de Mexico Federico Gomez, Ciudad de Mexico, Mexico
Presenting author: Ana V. Villarreal
P109 Corticosteroid-associated pneumatosis intestinalis in juvenile dermatomyositis: a case report
Bita Arabshahi1, John H. Lee2, Ian Leibowitz3
1Pediatric Rheumatology, Pediatric Specialists of Virginia, Fairfax, United States; 2Radiology, Fairfax Radiological Consultants PC, Fairfax, United States; 3Pediatric Gastroenterology, Pediatric Specialists of Virginia, Fairfax, United States
Presenting author: Bita Arabshahi
P110 Demographic and clinical characteristics of children with juvenile dermatomyositis in Cape Town
Lawrence O. Okong’o1, Jo Wilmshurst2, Monika Esser3, Christiaan Scott4
1Paediatric Rheumatology, University of Nairobi, Nairobi, Kenya; 2Paediatric Neurology, University of Cape Town, Cape Town, South Aftrica; 3Paediatric Immunology and Rheumatology, University of Stellenbosch, Cape Town, South Africa; 4Paediatric Rheumatology, University of Cape Town, Cape Town, South Africa
Presenting author: Christiaan Scott
P111 Pulmonary involvement in children with rheumatic diseases
Ezgi Deniz Batu1, Nagehan Emiroglu2, Hafize Emine Sonmez1, Gokcen Dilsa Tugcu2, Zehra Serap Arici1, Ebru Yalcin2, Deniz Dogru2, Ugur Ozcelik2, Yelda Bilginer1, Mithat Haliloglu3, Nural Kiper2, Seza Ozen1
1Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey; 2Department of Pediatrics, Division of Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey; 3Department of Radiology, Hacettepe University Faculty of Medicine, Ankara, Turkey
Presenting author: Ezgi Deniz Batu
P112 The clinical features of the juvenile dermatomyositis with anti-tif1 antibodies in Japan: report of three cases and review of the literature
Masato Yashiro, Mutsuko Yamada, Toshihiko Yabuuchi, Tomonobu Kikkawa, Nobuyuki Nosaka, Yosuke Fujii, Yukie Saito, Hirokazu Tsukahara
Pediatrics, Okayama University Hospital, Okayama, Japan
Presenting author: Masato Yashiro
P113 Juvenile dermatomyositis in Arab children: a multi-center retrospective analysis of disease features and outcome
Sulaiman M. Al-Mayouf1, Nora AlMutiari1,2, Mohammed Muzaffer3, Rawiah shehata4, Adel Al-Wahadneh5, Reem Abdwani6, Safia Al-Abrawi7, Mohammed Abu-shukair5, Zeyad El-Habahbeh5, Abdullah Alsonbul1
1King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia; 2King Faisal Specialist Hospital, Riyadh, Saudi Arabia; 3King Abdulaziz University, Jeddah, Saudi Arabia; 4Maternity & Children Hospital, Jeddah, Saudi Arabia; 5Queen Rania Children Hospital, Amman, Jordan; 6Qaboos Sultan University, Muscat, Oman; 7Royal Hospital, Muscat, Oman
Presenting author: Sulaiman M. Al-Mayouf
P114 Nailfold capillaroscopy in children with juvenile dermatomyositis
Aleksandra Szabat, Monika Chęć, Violetta Opoka-Winiarska
Department of Pediatric Pulmonology and Rheumatology, Medical University of Lublin, Poland, Lublin, Poland
Presenting author: Violetta Opoka-Winiarska
Poster Session: Scleroderma and related syndromes
P115 A single centre experience of pediatric systemic sclerosis from North India
Ankur Kumar1, Anju Gupta1, Amit Rawat1, Biman Saikia2, Ranjana W. Minz2, Deepti Suri1, Surjit Singh1
1Pediatrics, PGIMER, Chandigarh, India; 2Immunopathology, PGIMER, Chandigarh, India
Presenting author: Ankur Kumar
P116 Juvenile Localized Scleroderma (JLS): is it a benign disease?
Christine Arango1, Clara Malagon1, Maria D. P. Gomez2, Angela C. Mosquera1, Ricardo Yepez2, Tatiana Gonzalez3, Camilo Vargas4, and GRIP study group
1Pediatric Rheumatology, El Bosque University, Bogota, Colombia; 2Pediatric Rheumatology, Universidad Libre, Cali, Colombia; 3Pediatric Rheumatology, Universidad de Cartagena, Cartagena, Colombia; 4Pediatric Rheumatology, Universidad Del Valle, Cali, Colombia
Presenting author: Christine Arango
Circumscribed | Mixed | Linear | Generalized | CDS | Total | P value | |
---|---|---|---|---|---|---|---|
Frequency (%) | 29 | 28 | 12 | 10 | 7 | 86 | |
Mean age at diagnosis(years) | 8,0 | 6,2 | 8,1 | 7,3 | 5,9 | 6,9 | 0,255 |
Disease duration at diagnosis | 11,9 | 22,6 | 13,6 | 12,6 | 23,9 | 16,8 | 0,623 |
Extra cutaneous involvement n(%) | 2(6,9) | 7(25) | 3(25) | 2(20) | 3(42,9) | 17(19,8) | 0,194 |
Autoimmune associated disease n(%) | 4(3,8) | 4(14,3) | 2(16,7) | 1(10) | 0(0) | 11(12,8) | 0,854 |
Unsightly effects n(%) | 25(86,2) | 25(89,3) | 12(100) | 9(90) | 7(100) | 78(90,7) | 0,607 |
Joint functional involvement n(%) | 0(0) | 15(53,6) | 6(50) | 6(60) | 0(0) | 27(31,4) | 0,000 |
Growth disturbances n(%) | 3(10,3) | 17(60,7) | 7(58,3) | 2(20) | 5(71,4) | 34(39,5) | 0,000 |
2 or more complications n(%) | 3(10,3) | 19(67,9) | 7(58,3) | 7(70) | 5(71,4) | 41(47,7) | 0,001 |
P117 Magnetic resonance and echocardiographic strain rate imaging for the early detection of cardiac involvement in Juvenile Systemic Sclerosis
Francesco Zulian1, Marta Balzarin1, Biagio Castaldi2, Elena Reffo2, Francesca Sperotto1, Giorgia Martini1, Alessandra Meneghel1, Ornella Milanesi2
1Pediatric Rheumatology Unit, University of Padua, Padua, Italy; 2Pediatric Cardiology Unit, University of Padua, Padua, Italy
Presenting author: Francesco Zulian
P118 Update on the Juvenile Systemic Sclerosis inception cohort project. Characteristics of the first 80 patients at first assessment. Www.juvenile-scleroderma.com
Ivan Foeldvari1, Jens Klotsche2, Ozgur Kasapçopur3, Amra Adrovic3, Valda Stanevicha4, Maria Teresa Terreri5, Ekaterina Alexeeva6, Maria Katsicas7, Rolando Cimaz8, Mikhail Kostik9, Thomas Lehman10, W.-Alberto Sifuentes-Giraldo11, Vanessa Smith12, Flavio Sztajnbok13, Tadey Avcin14, Maria Jose Santos15, Dana Nemcova16, Cristina Battagliotti17, Despina Eleftheriou18, Liora Harel19, Mahesh Janarthanan20, Tilmann Kallinich2, Jordi Anton Lopez21, Kirsten Minden2, Susan Nielsen22, Kathryn Torok23, Yosef Uziel24, Nicola Helmus1
1Am Schön Klinik Eilbek, Hamburg, Germany; 2Pediatric Rheumatology, Berlin, Germany; 3Pediatric Rheumatology, Istanbul, Turkey; 4Pediatric Rheumatology, Riga, Latvia; 5Pediatric Rheumatology, Sao Paulo, Brazil; 6Pediatric Rheumatology, Moskva, Russian Federation; 7Pediatric Rheumatology, Buenos Aires, Argentina; 8Pediatric Rheumatology, Florence, Italy; 9Pediatric Rheumatology, St. Petersburg, Russian Federation; 10Pediatric Rheumatology, New York, United States; 11Pediatric Rheumatology, Madrid, Spain; 12Pediatric Rheumatology, Gent, Belgium; 13Pediatric Rheumatology, Rio de Janeiro, Brazil; 14Pediatric Rheumatology, Ljubljana, Slovenia; 15Pediatric Rheumatology, Almada, Portugal; 16Pediatric Rheumatology, Prague, Czech Republic; 17Pediatric Rheumatology, Santa Fee, Argentina; 18Pediatric Rheumatology, London, United Kingdom; 19Pediatric Rheumatology, Nettnja, Israel; 20Pediatric Rheumatology, Chennai, India; 21Pediatric Rheumatology, Barcelona, Spain; 22Pediatric Rheumatology, Copenhagen, Denmark; 23Pediatric Rheumatology, Pittsburgh, United States; 24Pediatric Rheumatology, Kfar Saba, Israel
Presenting author: Ivan Foeldvari
P119 Proposal of assessment of the activity of Juvenile Localised Scleroderma. Results of the consensus meeting in Hamburg, Germany December 2015
Ivan Foeldvari1, Eileen Baildem2, Michael Blakley3, Christina Boros4, Kim Fligelstone4, Antonia Kienast1, Dana Nemcova5, Clare Pain2, Amanda Saracino4, Gabriele Simoni6, Kathryn Torok7, Lisa Weibel8, Nicola Helmus1
1Am Schön Klinik Eilbek, Hamburg, Germany; 2Pediatric Rheumatology, Liverpool, United Kingdom; 3Pediatric Rheumatology, Indianapolis, United States; 4Pediatric Rheumatology, London, United Kingdom; 5Pediatric Rheumatology, Prague, Czech Republic; 6Pediatric Rheumatology, Firenze, Italy; 7Pediatric Rheumatology, Pittsburgh, United States; 8Pediatric Rheumatology, Zürich, Switzerland
Presenting author: Ivan Foeldvari
P120 Medication use in the Juvenile Systemic Sclerosis inception cohort. Are there differences in the diffuse and localised subset patients? Www.juvenile-scleroderma.com
Ivan Foeldvari1, Jens Klotsche2, Ozgur Kasapçopur3, Amra Adrovic3, Valda Stanevicha4, Maria Teresa Terreri5, Ekaterina Alexeeva6, Maria Katsicas7, Rolando Cimaz8, Mikhail Kostik9, Thomas Lehman10, W.-Alberto Sifuentes-Giraldo11, Vanessa Smith12, Flavio Sztajnbok13, Tadey Avcin14, Maria Jose Santos15, Dana Nemcova16, Cristina Battagliotti17, Despina Eleftheriou18, Liora Harel19, Mahesh Janarthanan20, Tilmann Kallinich2, Jordi Anton Lopez21, Kirsten Minden2, Susan Nielsen22, Kathryn Torok23, Yosef Uziel24, Nicola Helmus1
1Am Schön Klinik Eilbek, Hamburg, Germany; 2Pediatric Rheumatology, Berlin, Germany; 3Pediatric Rheumatology, Istanbul, Turkey; 4Pediatric Rheumatology, Riga, Latvia; 5Pediatric Rheumatology, Sao Paulo, Brazil; 6Pediatric Rheumatology, Moskva, Russian Federation; 7Pediatric Rheumatology, Buenos Aires, Argentina; 8Pediatric Rheumatology, Florence, Italy; 9Pediatric Rheumatology, St. Petersburg, Russian Federation; 10Pediatric Rheumatology, New York, United States; 11Pediatric Rheumatology, Madrid, Spain; 12Pediatric Rheumatology, Gent, Belgium; 13Pediatric Rheumatology, Rio de Janeiro, Brazil; 14Pediatric Rheumatology, Ljubljana, Slovenia; 15Pediatric Rheumatology, Almada, Portugal; 16Pediatric Rheumatology, Prague, Czech Republic; 17Pediatric Rheumatology, Santa Fee, Argentina; 18Pediatric Rheumatology, London, United Kingdom; 19Pediatric Rheumatology, Nettnja, Israel; 20Pediatric Rheumatology, Chennai, India; 21Pediatric Rheumatology, Barcelona, Spain; 22Pediatric Rheumatology, Copenhagen, Denmark; 23Pediatric Rheumatology, Pittsburgh, United States; 24Pediatric Rheumatology, Kfar Saba, Israel
Presenting author: Ivan Foeldvari
Number of patients | Whole group | Diffuse subtype | Limited subtype | 0.945 |
---|---|---|---|---|
Sex female /male | 80 | 57 (71%) | 23 (29%) | |
Medication | 66/14 = > 4.7:1 | 47/10 = > 4.7:1 | 20/4 = > 5:1 | |
62/71 (87%) | 44/50 (88%) | 18/21 (86%) | ||
Corticosteroids | 36/62 (58%) | 27/44 (61%) | 9/18 (50%) | 0.667 |
Cyclophosphamide | 5/62 (8%) | 5/44 (11%) | 0/18 (0%) | 0.159 |
Chloroquine/Hydroxy-chlorqquine | 10/62 (16%) | 6/44 (14%) | 4/18 (22%) | 0.485 |
Methotrexate | 35/62 (56%) | 24/44 (54%) | 11/18 (61%) | 0.804 |
Mycophenolate | 11/62 (18%) | 8/44 (18%) | 3/18 (17%) | 0.905 |
Azathioprin | 1/62 (2%) | 1/44 (2%) | 0/18 (0%) | 0.524 |
Tocolizumab | 1/62 (2%) | 0/44 (0%) | 1/18 (6%) | 0.125 |
Rituximab | 2/62 (3%) | 1/4 (2%) | 1/18 (6%) | 0.523 |
Bosentan | 11/62 (18%) | 10/44 (23%) | 1/18 (6%) | 0.165 |
PDE5 inhibitors | 4/62 (6%) | 3/4 (7%) | 1/18 (6%) | 0.863 |
Prostanoids | 1/62 (2%) | 0/44 (0%) | 1/18 (6%) | 0.125 |
P121 Cone beam computed tomograhy for evaluation of Craniofascial abnormalities in case of linear scleroderma en coup de sabre
Maria K. Osminina1, Nathalia A. Geppe1, Olga V. Niconorova2, Olesya V. Karashtina2, Oksana V. Abbyasova2, Olga V. Shpitonkova1
1Pediatric department, I.M.Sechenov First Moscow State Medical University, Moscow, Russian Federation; 2Pediatric department, Pediatric outpatien clinic of Public Joint-stock company Gazprom, Moscow, Russian Federation
Presenting author: Maria K. Osminina
P122 Role of pentraxin 3 in patients with juvenile scleroderma
Amra Adrovic1, Sezgin Sahin1, Kenan Barut1, Sinem Durmus2, Hafize Uzun2, Ozgur Kasapcopur1
1Pediatric Rheumatology, 2Biochemistry, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey
Presenting author: Amra Adrovic
P123 Is there a difference in the presentation of diffuse and limited subtype in childhood? Results from the juvenile scleroderma inception cohort. Www.juvenile-scleroderma.com
Ivan Foeldvari1, Jens Klotsche2, Ozgur Kasapçopur3, Amra Adrovic3, Valda Stanevicha4, Maria Teresa Terreri5, Ekaterina Alexeeva6, Maria Katsicas7, Rolando Cimaz8, Mikhail Kostik9, Thomas Lehman10, W.-Alberto Sifuentes-Giraldo11, Vanessa Smith12, Flavio Sztajnbok13, Tadey Avcin14, Maria Jose Santos15, Dana Nemcova16, Cristina Battagliotti17, Despina Eleftheriou18, Liora Harel19, Mahesh Janarthanan20, Tilmann Kallinich2, Jordi Anton Lopez21, Kirsten Minden2, Susan Nielsen22, Kathryn Torok23, Yosef Uziel24, Nicola Helmus1
1Am Schön Klinik Eilbek, Hamburg, Germany; 2Pediatric Rheumatology, Berlin, Germany; 3Pediatric Rheumatology, Istanbul, Turkey; 4Pediatric Rheumatology, Riga, Latvia; 5Pediatric Rheumatology, Sao Paulo, Brazil; 6Pediatric Rheumatology, Moskva, Russian Federation; 7Pediatric Rheumatology, Buenos Aires, Argentina; 8Pediatric Rheumatology, Florence, Italy; 9Pediatric Rheumatology, St. Petersburg, Russian Federation; 10Pediatric Rheumatology, New York, United States; 11Pediatric Rheumatology, Madrid, Spain; 12Rheumatology and Internal Medicine, Gent University Hospital, Gent, Belgium; 13Pediatric Rheumatology, Rio de Janeiro, Brazil; 14Pediatric Rheumatology, Ljubljana, Slovenia; 15Pediatric Rheumatology, Almada, Portugal; 16Pediatric Rheumatology, Prague, Czech Republic; 17Pediatric Rheumatology, Santa Fee, Argentina; 18Pediatric Rheumatology, London, United Kingdom; 19Pediatric Rheumatology, Nettnja, Israel; 20Pediatric Rheumatology, Chennai, India; 21Pediatric Rheumatology, Barcelona, Spain; 22Pediatric Rheumatology, Copenhagen, Denmark; 23Pediatric Rheumatology, Pittsburgh, United States; 24Pediatric Rheumatology, Kfar Saba, Israel
Presenting author: Ivan Foeldvari
Poster Session: Vasculitides
P124 Epidemiology of Kawasaki disease in Italy: surveillance from national hospitalization records
Angela Mauro1, Eleonora Fanti2, Fabio Voller2, Franca Rusconi3, Rolando Cimaz4
1Rheumatology Unit, Second University of Naples, Naples, Italy; 2Epidemiology Unit, Regional Health Agency of Tuscany, Florence, Italy; 3Epidemiology Unit, Anna Meyer Children’s University Hospital, Florence, Italy; 4Rheumatology Unit, Anna Meyer Children’s Hospital, Florence, Italy
Presenting author: Angela Mauro
P125 Is Harada Score a useful tool in Mexican children with Kawasaki disease?
Fernando Garcia-Rodriguez1, Ana V. Villarreal-Treviño1, Angel J. Flores-Pineda1, Paola B. Lara-Herrea1, Diego R. Salinas-Encinas1, Talia Diaz-Prieto1, Maria R. Maldonado-Velazquez1, Sarbelio Moreno-Espinosa2, Enrique Faugier-Fuentes1
1Pediatric Rheumatology, Hospital Infantil De Mexico Federico Gomez, Mexico City, Mexico; 2Pediatric Infectology, Hospital Infantil De Mexico Federico Gomez, Mexico City, Mexico
Presenting author: Fernando Garcia-Rodriguez
P126 Behçet disease in the pediatric age: data on 129 patients collected from an Italian cohort
Romina Gallizzi1, Martina Finetti2, Mirella Crapanzano1, Luca Cantarini3, Marco Cattalini4, Giovanni Filocamo5, Antonella Insalaco6, Angela Mauro7, Donato Rigante8, Francesco Zulian9, Maria Alessio10, Ilaria Parissenti4, Nicolino Ruperto2, Marco Gattorno2, Rolando Cimaz11
1Aou G.Martino, Messina, Italy; 2Istituto Gaslini, Genova, Italy; 3Policlinico Le Scotte, Siena, Italy; 4Clinica Pediatrica, Brescia, Italy; 5Ospedale Maggiore, Clinica Pediatrica II De Marchi, Milano, Italy; 6Ospedale Bambin Gesù, Roma, Italy; 7II Università degli Studi di Napoli, Napoli, Italy; 8Policlinico Gemelli, Roma, Italy; 9Ospedale Salus Pueri, Padova, Italy; 10Ospedale Federico II, Napoli, Italy; 11Ospedale Meyer, Firenze, Italy
Presenting author: Romina Gallizzi
P127 Endothelial dysfunction in children with Kawasaki disease and transient coronary artery abnormalities
Man S. Parihar1, Surjit Singh1, Pandiarajan Vignesh1, Anju Gupta1, ManojKumar Rohit2
1Allergy Immunology Unit, Advanced Pediatrics Centre, Chandigarh, India; 2Cardiology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Presenting author: Surjit Singh
P128 Carotid intima-media thickness and lipid profile in children with Kawasaki disease: a follow-up study after 5 years
Kavitha Gopalan1, Surjit Singh1, Pandiarajan Vignesh1, Anju Gupta1, ManojKumar Rohit2, Savita V. Attri3
1Allergy immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India; 2Cardiology, Post Graduate Institute of Medical Education and Research, Chandigarh, India; 3Division of Biochemistry, Advanced Pediatrics Centre, Chandigarh, India
Presenting author: Surjit Singh
P129 Microparticles as a therapeutic target for plasma exchange in antineutrophil cytoplasmic antibody associated vasculitis?
Ying Hong1, Despina Eleftheriou1, Sira Nanthapisal1, Alan Salama2, David Jayne3, Mark Little4, Paul Brogan1
1Infection, Immunity, Inflammation and Physiological Medicine, Institute of Child Health, UCL, London, UK; 2UCL Centre for Nephrology, Royal Free Hospital, London, UK; 3Vasculitis and Lupus Clinic, Addenbrookes Hospital, Cambridge, UK; 4School of Medicine, Trinity College Dublin, Dublin, United Kingdom
Presenting author: Ying Hong
P130 Takaysu arteritis in children: experience in observation and treatment of a single center
Yulia Kostina1, Galina Lyskina1, Olga Shpitonkova1
1Pediatric Department, I.M. Sechenov First Moscow State Medical University, Moscow, Russian Federation
Presenting author: Yulia Kostina
P131 The age features of Kawasaki disease, Moscow, Russian Federation
Alena Torbyak, Galina Lyskina, Olga Shirinsky
Department of Childhood diseases, I.M.Sechenov First Moscow State Medical University, Moscow, Russian Federation
Presenting author: Alena Torbyak
P132 Early onset of behcet disease: a case report
Angela Mauro1, Maria Francesca Gicchino1, Maria Cristina Smaldone1, Mario Diplomatico1, Alma Nunzia Olivieri1
1Rheumatology Unit, Second University of Naples, Naples, Italy
Presenting author: Angela Mauro
P133 A quality improvement initiative to improve urinalysis follow-up in henoch-schonlein purpura: retrospective review and prospective intervention
C H. Spencer1, Rabheh Abdul Aziz2, Richard McClead2, Sharon Bout-Tabaku2, Hiren Patel3, Chung-Yung Yu2
1Rheumatology, Nationwide Childrens/Ohio State, Columbus OH, USA; 2Rheumatology, Nationwide Childrens/Ohio State, Columbus, United States; 3Nephrology, Nationwide Childrens/Ohio State, Columbus, United States
Presenting author: C H. Spencer
P134 Successful treatment of digital necrotizing vasculitis in patients with polyarteritis nodosa
Coskun F. Ozkececi1, Gokalp Basbozkurt2, Dogan Simsek2, Esra Kurt2, Faysal Gok2, Erkan Demirkaya2
1Pediatrics, Gulhane Military Medical Academy, Ankara, Turkey; 2Pediatric, Gulhane Military Medical Academy, Ankara, Turkey
Presenting author: Coskun F. Ozkececi
P135 Paediatric large vessel vasculitis presenting as idiopathic retroperitoneal fibrosis: case report and review of the literature
Dita Cebecauerová1, Tomáš Dallos2, Edita Kabíčková3, Martin Kynčl4, Daniela Chroustová5, Jozef Hoza1, Dana Němcová1, Vladimír Tesař6, Pavla Doležalová1
1Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic; 2Pediatric Rheumatology Service, 2nd Department of Pediatrics, Comenius University Medical School and University Children’s Hospital, Bratislava, Slovakia; 3Department of Pediatric Hematology and Oncology, Charles University and University Hospital Motol, Prague, Czech Republic; 4Department of Radiology, Second Faculty of Medicine and University Hospital Motol, Prague, Czech Republic; 5Department of Nuclear Medicine, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic; 6Department of Nephrology, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic
Presenting author: Dita Cebecauerová
P136 Tocilizumab treatment in childhood Takayasu arteritis: case series of four patients and systematic review of the literature
Ezgi Deniz Batu1, Hafize Emine Sonmez1, Tuncay Hazirolan2, Fatih Ozaltin3, Yelda Bilginer1, Seza Ozen1
1Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey; 2Department of Radiology, Hacettepe University Faculty of Medicine, Ankara, Turkey; 3Department of Pediatrics, Division of Nephrology, Hacettepe University Faculty of Medicine, Ankara, Turkey
Presenting author: Ezgi Deniz Batu
P137 Neurological manifestation in Behçet’s disease- pediatric case report
Fabiola Almeida1, Isabela H. Faria de Paula1, Maíra M. Sampaio2, Fernando N. Arita2, Andressa G. Alves1, Maria Carolina Santos1, Eunice M. Okuda1, Silvana B. Sacchetti1
1Pediatric Rheumatology, Irmandade da Santa Casa de Misericórdia de São Paulo- Brasil, São Paulo, Brazil; 2Pediatric Neurology, Irmandade da Santa Casa de Misericórdia de São Paulo- Brasil, São Paulo, Brazil
Presenting author: Fabiola Almeida
P138 Vitamin d receptor polymorphisms are not associated with the risk of Kawasaki disease (KD) in a group of Italian children
Fernanda Falcini1, Marini Francesca2, Stefano Stagi3, Donato Rigante4, Gemma Lepri5, Marco Matucci-Cerinic1, Maria Luisa Brandi2
1Dept. of Internal Medicine, Section of Rheumatology, Transition Clinic; 2Dept. of Surgery and Translational Medicine; 3Health Sciences Department, Anna Meyer Children’s University Hospital, University of Florence, Florence, Italy; 4Institute of Pediatrics, Università Cattolica Sacro Cuore Universityy of Rome, Rome, Italy; 5Dept. of Internal Medicine, Section of Rheumatology, Transition Clinic, University of Florence, Florence, Italy
Presenting author: Fernanda Falcini
P139 The proteiform manifestations of Behçet disease. Description of 2 cases with prominent gastrointestinal and renal involvement
Giancarla Di Landro1, Sofia Torreggiani1, Antonella Petaccia1, Marta Torcoletti1, Fabrizia Corona1, Giovanni Filocamo1
1Dipartimento della Donna, del Bambino o del Neonato, Fondazione Irccs Cà Granda Ospedale Maggiore Policlinico, Milano, Italy
Presenting author: Giancarla Di Landro
P140 Assessment of relationship between IL-17 levels and oxidative stress in children with immunoglobulin a vasculitis
Hakan Kisaoglu1, Sema Misir2,3, Selim Demir4, Yuksel Aliyazicioglu3, Mukaddes Kalyoncu5
1Department of Pediatrics, Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey; 2Department of Biochemistry, Cumhuriyet University Faculty of Pharmacy, Sivas, Turkey; 3Department of Medical Biochemistry, Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey; 4Department of Nutrition and Dietetics, Karadeniz Technical University, Faculty of Health Sciences, Trabzon, Turkey; 5Department of Pediatric Rheumatology, Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey
Presenting author: Hakan Kisaoglu
P141 Associatiom between pediatric cutaneous polyarteritis nodosa and streptococcal infection - a single tertiary center experience
Isabela H. Faria de Paula1, Carlos Eduardo Ramalho2, Fabiola D. Almeida1, Andressa G. Alves1, Maria Carolina Santos1, Silvana B. Sacchetti1, Eunice M. Okuda1
1Pediatric Rheumatology, Irmandade Santa Casa de Misericordia de São Paulo, São Paulo, Brazil; 2Pediatric, Irmandade Santa Casa de Misericordia de São Paulo, São Paulo, Brazil
Presenting author: Isabela H. Faria de Paula
P142 Abciximab for treatment of coronary aneurysms in Kawasaki disease
Joan Calzada-Hernández1, Rosa Bou1, Estíbaliz Iglesias1, Judith Sánchez-Manubens1, Fredy Hermógenes Prada Martínez2, Clara Giménez Roca1, Sergi Borlan Fernández1, Juan Manuel Mosquera Angarita1, Jordi Anton1
1Pediatric Rheumatology Unit, Department of Pediatrics, Hospital Sant Joan de Déu, Esplugues de Llobregat (Barcelona), Spain; 2Department of Pediatric Cardiology, Hospital Sant Joan de Déu, Esplugues de Llobregat (Barcelona), Spain
Presenting author: Joan Calzada-Hernández
P143 Cogan syndrome presented in 5 years old girl with history of hypoglycaemic episodes and with significant behavioral changes
Marek Bohm1, Kamran Mahmood1, Valentina Leone1, Mark Wood2
1Paediatric Rheumatology, Leeds General Infirmary, Leeds Teaching Hospitals Trust, Leeds, UK, Leeds, United Kingdom; 2Paediatric Rheumatology, Leeds General Infirmary, Leeds Teaching Hospitals Trust, Leeds, UK, Prague, Czech Republic
Presenting author: Kamran Mahmood
P144 Sensitivity of four criteria for Behçet’s disease in Japanese pediatric patients
Ken-Ichi Yamaguchi1, Satoshi Fujikawa1,2, and Working Group of Behçet’s Disease, Pediatric Rheumatology Association of Japan (PRAJ)
1Immuno-Rheumatology Center, St. Luke’s International Hospital, Tokyo, Japan; 2Fujikawa Pediatric Clinic, Tokyo, Japan
Presenting author: Ken-Ichi Yamaguchi
P145 Increased serum antibody titer against HPV-16 antigen in patients with Behçet’s disease
Kyu Yeun Kim1, Do Young Kim2, Dong Soo Kim1
1Pediatrics, Division of Rheumatology, College of Medicine, Yonsei University, Severance Children’s Hospital, Seoul, Korea, Republic Of; 2Department of Dermatology, College of Medicine, Yonsei University, Seoul, Korea, Republic Of
Presenting author: Kyu Yeun Kim
P146 A case of Churg-Strauss syndrome in a 12-year-old girl
Maka Ioseliani, Ivane Chkhaidze, Maia Lekishvili, Nana Tskhakaia, Shorena Tvalabeishvili, Aleksandre Kajrishvili
M. Iashvili Children’s Central Hospital, Tbilisi, Georgia
Presenting author: Maia Lekishvili
P147 Successful treatment with etanercept in a patient with refractory cutaneous polyarteritis nodosa
Maiko Takakura, Masaki Shimizu, Natsumi Inoue, Mao Mizuta, Akihiro Yachie
Department of Pediatrics, School of Medicine, Institute of Medical Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan
Presenting author: Maiko Takakura
P148 Complicated schoenlein-hoenoch with severe organ involvement: personal records
Clotilde Alizzi, Giovanni Corsello, Maria Cristina Maggio
University Department Pro.Sa.M.I. “G. D’Alessandro”, University of Palermo, Palermo, Italy
Presenting author: Maria Cristina Maggio
P149 Incidence of IgA Vasculitis in children: A population-based study using a four-source capture recapture estimate
Maryam Piram1,2,3, Carla Maldini4, Sandra Biscardi5, Nathalie Desuremain6, Catherine Orzechowski7, Emilie Georget8, Delphine Regnard9, Isabelle Kone-Paut10,11, Alfred Mahr4,12
1Inserm 1018, CESP, Kremlin Bicêtre, Paris, France; 2Pediatric Rheumatology, CHU de Bicêtre, Paris, France; 3Université Paris Sud, Le Kremlin Bicêtre, Paris, France; 4Internal Medecine, CHU Saint Louis, Paris, France; 5Pediatrics, CHIC, Créteil, Paris, France; 6Pediatrics, CHU Trousseau, Paris, France; 7Pediatrics, CH Sainte Camille, Bry sur Marne, Paris, France; 8Pediatrics, CHIV, Villeneuve Saint Georges, Paris, France; 9Pediatrics, CHU de Bicêtre, Paris, France; 10Pediatric Rheumatology, CHU de Bicêtre, Paris, France; 11Université Paris Sud, Kremlin Bicêtre, Paris, France; 12Université Paris 7-Diderot, ECSTRA, Paris, France
Presenting author: Maryam Piram
P150 Severe systemic vasculitis associated with primary immunodeficiency in two siblings
Mihaela Sparchez1, Laura Damian2, Zeno Sparchez3
12nd Department of Paediatrics, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj Napoca, Romania; 2Rheumatology Department, Emergency Clinical County Hospital, Cluj Napoca, Romania; 33rd Department of Internal Medicine, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj Napoca, Romania
Presenting author: Mihaela Sparchez
P151 Henoch-Schönlein purpura, a general view about the more often vasculitis in pediatric population and a study of forecast factors of renal disease relapse in Mexican population
Nydia Acevedo Silva, Ana V. Villarreal Treviño, Yuridiana Ramirez Loyola, Talia Diaz Prieto, Enrique Faugier Fuentes, Maria D. R. Maldonado Velazquez
Pediatric Rheumatology, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico
Presenting author: Nydia Acevedo Silva
Characteristics | All patients | Patients with Nephritis | Patients without Nephritis | Value of P | |
---|---|---|---|---|---|
N = 79 | n = 17 | n = 62 | |||
Elevated creatinine | Yes | 5% | 22.2% | 1.4% | ≤0.001 |
No | 95% | 77.8% | 98.6% | ||
12 hrs proteinuria mg/m2/hra | 4.57 ± 7.65 | 10.47 ± 13 | 2.9 ± 2.3 | ≤0.001 | |
Proteinuria | Yes | 63% | 88% | 23% | ≤0.001 |
No | 37% | 12% | 76% | ||
Relapse | Yes | 19.5% | 50% | 11.6% | ≤0.001 |
No | 80.5% | 50% | 88% |
P152 Prevalence of kidney involvement during the first follow-up year in patients with Henoch–Schönlein Purpura in a pediatric institution in Bogota, Colombia
Pilar Perez, Angela C. Mosquera, Clara Malagon
Pediatric Rheumatology, El Bosque University, Bogota, Colombia
Presenting author: Pilar Perez
P153 T helper 17 (Th17) and T regulatory (Treg) cells in children with Kawasaki disease – experience from a tertiary care centre in North India
Sagar Bhattad1, Amit Rawat1, Biman Saikia2, Ranjana Minz2, Jitendra Shandilya1, Surjit Singh1 and Pediatric Allergy and Immunology Unit, PGIMER, Chandigarh
1Pediatrics, PGIMER, Chandigarh, India; 2Immunopathology, PGIMER, Chandigarh, India
Presenting author: Sagar Bhattad
Cases (Pre-IVIg) | Cases (Post-IVIg) | Febrile controls | Controls | |
---|---|---|---|---|
Th17 cells | 2.58% | 1.88% | 1.57% | 2% |
Treg cells | 0.99% | 2.64% | 1.96% | 2.37% |
P154 QT dispersion in children with Kawasaki disease with transient coronary artery abnormalities: a follow-up study
Man S. Parihar1, Surjit Singh1, Pandiarajan Vignesh1, Anju Gupta1, ManojKumar Rohit2
1Allergy immunology Unit, Advanced Pediatrics Centre, Chandigarh, India; 2Cardiology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Presenting author: Surjit Singh
P155 Infliximab treatment for a patient with giant coronary aneurysm with refractory Kawasaki disease: 8-month old male. Case report and brief review of literature
Rocio Maldonado1, Enrique Faugier1, Ana Villarreal1, Nydia Acevedo1, Yuridiana Ramírez1, Talia Diaz1
1Paediatric Rheumatology, Hospital Infantil de México Federico Gómez, Mexico City, Mexico
Presenting author: Enrique Faugier
P156 Fibromuscular dysplasia in the practice of child rheumatologist
Yulia Kostina, Galina Lyskina, Olga Shpitonkova
Pediatric Department, I.M. Sechenov First Moscow State Medical University, Moscow, Russian Federation
P157 Is neutrophil to lymphocyte ratio valid to predict organ involvement in Henoch-Schönlein Purpura?
Kubra Ozturk1, Zelal Ekinci1
1Department of Pediatrics Rheumatology, Kocaeli Univertsity Faculty of Medicine, Kocaeli, Turkey
Presenting author: Zelal Ekinci
P158 Childhood polyarteritis nodosa: diagnosis with noninvasive imaging techniques
Zeynep Birsin Özçakar1, Suat Fitoz2, Fatos Yalcinkaya1
1Pediatric Rheumatology, Ankara University, Ankara, Turkey; 2Pediatric Radiology, Ankara University, Ankara, Turkey
Presenting author: Zeynep Birsin Özçakar
Poster Session: Systemic JIA I
P159 Factors associated with etoposide usage in children with macrophage activation syndrome complicating systemic Juvenile Idiopathic Arthritis
Annacarin Horne1, Francesca Minoia2, Francesca Bovis2, Sergio Davi2, Priyankar Pal3, Jordi Anton4, Kimo Stein5, Sandra Enciso6, Ozgur Kasapcopur7, Michael Jeng8, Despoina Maritsi9, Randy C. Cron10, Angelo Ravelli11
1Peadiatric Rheumatology, Karolinska Institutet, Stockholm, Sweden; 2Peadiatric rheumatology, Instituto Giannina Gaslini, Genoa, Italy; 3Peadiatric Rheumatology, Institute of Child Health, Kolkata, India; 4Peadiatric rheumatology, Hospital Sant Joan de Deu, Barcelona, Spain; 5Pediatric oncology, Arkansas Children’s Hospital, Little Rock, United States; 6Pediatric Oncology, Hospital Infantil de Mexico City, Mexico City, Mexico; 7Pediatric Rheumatology, University of Istanbul, Istanbul, Turkey; 8Pediatric Oncology, Stanford, Palo Alto, United States; 9Pediatric Rheumatology, Athens Medical School, Athens, Greece; 10Pediatric Rheumatology, University of Alabama at Birmingham, Birmingham, United States; 11Pediatric Rheumatology, Instituto Giannina Gaslini, Genoa, Italy
Presenting author: Annacarin Horne
P160 A novel homozygous frameshift mutation in LACC1 associated with severe familial forms of Juvenile Idiopathic Arthritis
Anne Thorwarth1, Sae Lim von Stuckrad1, Angela Rösen-Wolff2, Hella Luksch2, Patrick Hundsdoerfer3, Kirsten Minden4, Peter Krawitz5, Tilmann Kallinich1
1Pediatric Pneumology and Immunology, Charité University Medicine Berlin, Berlin, Germany; 2Department of Pediatrics, University Clinic Carl Gustav Carus, Dresden, Germany; 3Pediatric Oncology and Hematology, Charité University Medicine Berlin, Berlin, Germany; 4Department of Rheumatology and Clinical Immunology, Charité University Medicine Berlin, Berlin, Germany; 5Institute of Medical Genetics and Human Genetics, Charité University Medicine Berlin, Berlin, Germany
Presenting author: Anne Thorwarth
P161 The systemic onset Juvenile Idiopathic Arthritis national registry in Turkey: a preliminary report
Betul Sozeri1, Nuray Aktay ayaz2, Ezgi Deniz Batu3, Balahan Makay4, Sezgin Şahin5, Doğan Simsek6, Şebnem Sara Kılıc7, Kubra Ozturk8, Emine Sonmez3, Aysenur Pac Kisaarslan1, Ozge Altug Gucenmez4, Mustafa Cakan2, Z. Serap Arıcı3, Amra Adrovic5, Fatih Kelesoglu9, Yelda Bilginer3, Erkan Demirkaya6, Zelal Ekinci Ekinci8, Ruhan Dusunsel1, Erbil Unsal4, Ozgur Kasapcopur5, Seza Ozen3
1Pediatric Rheumatology, Erciyes University Faculty of Medicine, Kayseri, Turkey; 2Pediatric Rheumatology, Istanbul Kanuni Sultan Suleyman Education and Research Hospital, Istanbul, Turkey; 3Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey; 4Pediatric Rheumatology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey; 5Pediatric Rheumatology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey; 6Pediatric Rheumatology, Gulhane Military Medical Faculty, FMF Arthritis Vasculitis and Orphan disease Research Center, Ankara, Turkey; 7Pediatric Rheumatology, Uludag University Faculty of Medicine, Bursa, Turkey; 8Pediatric Rheumatology, Kocaeli University Faculty of Medicine, Kocaeli, Turkey; 9Pediatric Rheumatology, Capa Medical Faculty, Istanbul University, Istanbul, Turkey
Presenting author: Betul Sozeri
P162 Serial serum interleukin-6 levels in systemic Juvenile Idiopathic Arthritis as a predictor of treatment response
Butsabong Lerkvaleekul, Soamarat Vilaiyuk
Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
Presenting author: Butsabong Lerkvaleekul
P163 Validation of MRP8/14 serum levels as biomarker for the diagnosis of systemic Juvenile Idiopathic Arthritis in fever of unknown origin
Maria Miranda-Garcia1, Carolin Pretzer1, Hans-Iko Huppertz2, Gerd Horneff3, Johannes-Peter Haas4, Gerd Ganser5, Jasmin Kuemmerle-Deschner6, Helmut Wittkowski1, Michael Frosch7, Johannes Roth8, Dirk Foell1, Dirk Holzinger1
1Department of Pediatric Rheumatology and Immunology, University Children’s Hospital Muenster, Muenster, Germany; 2Prof.-Hess Children’s Hospital and Gesundheit Nord Klinikverbund Bremen, Bremen, Germany; 3Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany; 4German Center for Pediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany; 5St. Josef-Stift Sendenhorst Hospital, Sendenhorst, Germany; 6Department of Pediatrics, Division of Pediatric Rheumatology, University Children’s Hospital Tuebingen, Tuebingen, Germany; 7German Pediatric Pain Centre, Children’s and Adolescents’ Hospital, Datteln, Germany; 8Institute of Immunology, University Hospital Muenster, Muenster, Germany
Presenting author: Dirk Holzinger
P164 Proteomic discovery of SJIA diagnostic and phenotypic biomarkers
F Gohar1, Angela McArdle2, Niamh Callan2, Belinda Hernandez2, Miha Lavric1, Christoph Kessel1, Dirk Holzinger1, Oliver FitzGerald2, Stephen R. Pennington2, Dirk Foell1
1Paediatric Rheumatology and Immunology, University of Muenster, Muenster, Germany; 2UCD Conway Institute of Biomolecular and Biomedical Research, University College Dublin, Dublin, Ireland
Presenting author: F Gohar
Univariate analysis | Multivariate analysis: AUC | Overlapping proteins | |
---|---|---|---|
Differentially expressed proteins, n, p ≤ 0.05 | (Top 50 differentially expressed proteins) | (n) | |
Sys_SJIA vs artic_SJIA | 41 | 0.94 | 5 |
SJIA (all) vs infection | 35 | 0.86 | 3 |
Sys_SJIA vs infection | 31 | 0.58 | 2 |
Artic_SJIA vs infection | 36 | 0.97 | 4 |
P165 Anakinra for first line steroid free treatment in systemic onset Juvenile Idiopathic Arthritis
Gerd Horneff1, Joachim Peitz1, Joern Kekow2, Ariane Klein1
1ASKLEPIOS, Sankt Augustin, Germany, 2Helios Clinic, Vogelsang, Germany
Presenting author: Gerd Horneff
Patient | 1 | 2 | 3 | 4 | 5 | 6 |
---|---|---|---|---|---|---|
Age (years), gender | 11, m | 2, m | 1, f | 8, f | 15, f | 11, m |
Systemic features | Spiking fever, rash, | Spiking fever, rash, hepatomegaly, splenomegaly | Spiking fever, rash, hepatomegaly, splenomegaly | Spiking fever, rash | Spiking fever, rash | Spiking fever, rash, hepatomegaly, splenomegaly |
Joint involvement | Arthralgia | Arthralgia | Oligoarthritis | Poly-arthritis | Oligo-arthritis | Arthralgia |
ESR, mm/h | 45 | 68 | na | na | 63 | na |
CRP, mg/l | 95.6 | 101.7 | 106.6 | 197.0 | 329.8 | 99.0 |
IL18, pg/ml | 2,483 | >10,000 | >5,000 | >10,000 | >10,000 | >5,000 |
Pretreatment | 3 days corticosteroids | none | none | none | none | 3 days corticosteroids |
Initial response | Immediate complete response | Immediate complete response | Immediate partial response | none | Improvement of arthritis, suppression of fever | Immediate complete response |
Outcome | Remission off treatment | Remission off treatment | Increase of dosage upon flare, discontinued with MAS | No response, switch to Tocilizumab | Minor response, switch to Tocilizumab | Remission, relapse after discontinuation |
P166 Experience with Tocilizumab, interleukin-1 inhibitors and etanercept for systemic Juvenile Idiopathic Arthritis
Gerd Horneff1, Anna C. Schulz1, Kirsten Minden2, Frank Weller-Heinemann3, Anton Hospach4, J-Peter Haas5, and BIKER collaborative group
1ASKLEPIOS, Sankt Augustin, Germany; 2Charite, Berlin, Germany; 3Prof. Hess Children’s Hospital, Bremen, Germany; 4Olgahospital, Stuttgart, Germany; 5German Centre Pediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany
Presenting author: Gerd Horneff
Etanercept | Tocilizumab | IL-1i | ||
---|---|---|---|---|
N = 143 | n = 72 | N = 55 | ||
Baseline | JADAS10 Median (IQR) | 8.2 (5.3–13.0) | 9.6 (4.9–13.3) | 8.2 (5.1–13.3) |
Month 6 | JADAS10 Median (IQR) | 6.2(1.1–15.6) | 0.8 (0.1–5.3) | 6.6 (0.2–2.0) |
JADAS MDA n (%) | 26 (29.5%) | 21 (61.8%) | 21 (65.6%) | |
JADAS Remission n (%) | 16 (18.2%) | 15 (44.1%) | 16 (50%) | |
Discontinuations (< Month 6) | Inefficacy (n, %) | 23 (26.1%) | 4 (11.8%) | 7 (21.9%) |
Intolerance (n, %) | 1 (2.9%) | |||
Remission (n, %) | 1 (1.1%) |
P167 Inflammatory gene expression profile and defective interferon-gamma and granzyme k in natural killer cells of systemic Juvenile Idiopathic Arthritis patients
Karen Put1, Jessica Vandenhaute1, Anneleen Avau1, Annemarie van Nieuwenhuijze2, Ellen Brisse1, Tim Dierckx3, Omer Rutgeerts4, Josselyn E. Garcia-Perez2, Jaan Toelen5, Mark Waer4, Georges Leclercq6, An Goris7, Johan Van Weyenbergh3, Adrian Liston2, Lien De Somer8, Patrick Matthys1, Carine H. Wouters8,9
1Laboratory of Immunobiology, Rega Institute, University of Leuven, Leuven, Belgium; 2Autoimmune Genetics Laboratory, VIB, Ghent, Belgium; 3Laboratory of Clinical and Epidemiological Virology, Rega Institute, University of Leuven, Leuven, Belgium; 4Laboratory of Experimental Transplantation, KU Leuven - University of Leuven, Leuven, Belgium; 5Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium; 6Department of Clinical Chemistry, Ghent University, Ghent, Belgium; 7Laboratory of Neuroimmunology, KU Leuven - University of Leuven, Leuven, Belgium; 8Pediatric Rheumatology, University Hospitals Leuven, Leuven, Belgium; 9Laboratory of Pediatric Immunology, KU Leuven - University of Leuven, Leuven, Belgium
Presenting author: Jessica Vandenhaute
P168 Serum IL-18 levels as a marker for disease activity in systemic Juvenile Idiopathic Arthritis during tocilizumab therapy
Mao Mizuta1, Masaki Shimizu1, Natsumi Inoue1, Yasuo Nakagishi2, Akihiro Yachie1
1Department Of Pediatrics, School Of Medicine, Institute of Medical Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan; 2Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Japan
Presenting author: Mao Mizuta
P169 Cytokine profile of Adult-Onset Still’s Disease: comparison with systemic Juvenile Idiopathic Arthritis
Masaki Shimizu, Natsumi Inoue, Mao Mizuta, Akihiro Yachie
Department of Pediatrics, School of Medicine, Institute of Medical Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan
Presenting author: Masaki Shimizu
P170 Genetic architecture of systemic Juvenile Idiopathic Arthritis distinguishes it from oligo- and polyarticular Juvenile Idiopathic Arthritis
Michael J. Ombrello1, Victoria Arthur1, Elaine F. Remmers2, Anne Hinks3, Daniel L. Kastner2, Patricia Woo4, Wendy Thomson3,5, and International Childhood Arthritis Genetics (INCHARGE) Consortium
1Tranlational Genetics and Genomics Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, United States; 2Inflammatory Disease Section, National Human Genome Research Institute, Bethesda, United States; 3Arthritis Research UK Centre for Genetics and Genomics, Manchester Academic Health Science Centre, Manchester, UK; 4Center of Paediatric and Adolescent Rheumatology, University College London, London, UK; 5NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom
Presenting author: Michael J. Ombrello
P171 Bone marrow findings at children with Juvenile Idiopathic Arthritis
Barbara Stanimirovic1, Biljana Djurdjevic-Banjac2, Olivera Ljuboja3
1Pediatric Rheumatology, University Clinical Centar Republic of Srpska, Banjaluka, Bosnia and Herzegovina; 2Pediatric Hematology, University Clinical Centar Republic of Srpska, Banjaluka, Bosnia and Herzegovina; 3Pediatric Pulmology, University Clinical Centar Republic of Srpska, Banjaluka, Bosnia and Herzegovina
Presenting author: Barbara Stanimirovic
P172 Inflammatory bowel disease following anti-interleukin-1-treament in systemic Juvenile Idiopathic Arthritis
Boris Hugle, Fabian Speth, Johannes-Peter Haas
German Center for Pediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany
Presenting author: Boris Hugle
P173 Complete clinical remission with tocilizumab in two toddlers with systemic Juvenile Idiopathic Arthritis-a case series
Despoina Maritsi1, MArgarita Onoufriou2, Olga Vougiouka3, Despina Eleftheriou4
1Pediatrics, P & A Kyriakou Children;s Hospital, Athens, Greece; 2Pediatrics, Makarios III Children;s Hospital, Nicosia, Cyprus; 3Pediatrics, P & A Kyriakou Children’s Hospital, Athens, Greece; 4Paediatric and Adolescent Rheumatology, Great Ormond Street Hospital, London, United Kingdom
Presenting author: Despoina Maritsi
Patient characteristics | Patient I | Patient II |
---|---|---|
Age | 11 months | 18 months |
Ethnicity | Caucasian | Caucasian |
Gender | male | female |
Disease duration | 3 months | 7 months |
Steroid dose | 2 mg/kg/day | 1 mg/kg/day |
Methotrexate dose (SC) | 15 mg/m2
| 15 mg/m2
|
Tocilizumab dose (IV) | 12 mg/kg every two weeks | 12 mg/kg every two weeks |
ACR Pedi 30 (+ no fever) | By week 12 | By week 8 |
ACR Pedi 90 (+no fever) | By week 36 | By week 24 |
Adverse events | Varicella | Recurrent upper respiratory tract infections |
P174 Canakinumab for first line steroid free treatment in a child with systemic onset Juvenile Idiopathic Arthritis
Gerd Horneff1, Joachim Peitz1, Joern Kekow2, Dirk Foell3
1ASKLEPIOS, Sankt Augustin, Germany, 2Helios, Vogelsang, Germany, 3University, Muenster, Germany
Presenting author: Gerd Horneff
HB (g/dl) [11.5–13.5] | CRP (mg/l) [<5] | Leukocytes (/μl) [5,000–14,500] | Thrombo-cytes (/nl) [150–450] | ASAT (U/l) [<38] | LDH /U/l) [155–345] | Ferritin (μg/l) [7–142] | IL-18 (pg/ml) [50–300] | S100A8/A9 (ng/ml) [<2,940) | |
---|---|---|---|---|---|---|---|---|---|
Presentation (day -20) | 9.6 | 60 | 18,800 | n | n | n | 4,409 | ||
Baseline | 8.8 | 166.12 | 7,360 | 54 | 119 | 1028 | >1,200 | >10,000 | 134,000 |
Day 1 | 8.6 | 41.52 | 6,470 | 85 | 124 | 852 | |||
Day 3 | 9.1 | 21.35 | 6,820 | 82 | 69 | 520 | 24,000 | ||
Day 22 | 11.3 | 0.11 | 8,720 | 419 | 38 | 278 | 1,773 | ||
Day 50 | 10.9 | 0.27 | 7,080 | 316 | 34 | 286 | 63 | 2,805 | 1,150 |
Month 3 | 12.1 | 0.06 | 7,650 | 254 | 34 | 255 |