Skip to main content
Erschienen in: Clinical Rheumatology 3/2019

31.01.2019 | Original Article

Frequency of functional gastrointestinal disorders in children with familial Mediterranean fever

verfasst von: Rabia Miray Kisla Ekinci, Sibel Balcı, Eray Akay, Gokhan Tumgor, Dilek Dogruel, Derya Ufuk Altintas, Mustafa Yilmaz

Erschienen in: Clinical Rheumatology | Ausgabe 3/2019

Einloggen, um Zugang zu erhalten

Abstract

Introduction

Familial Mediterranean fever (FMF) is characterized by self-limiting fever episodes usually accompanied by serositis, arthralgia, and arthritis. Functional gastrointestinal disorders (FGIDs) are diseases in which brain-gut axis and low-grade inflammation take part in pathogenesis. We aimed to study the FGIDs frequencies and possible risk factors for FGIDs in children with FMF.

Method

This case-control study included 103 children with FMF followed up between July 2016 and July 2018 and 100 healthy controls. Age, gender, disease characteristics, and MEFV gene results were recorded retrospectively. Laboratory parameters were obtained at the time of study enrollment. Diagnosis of FGIDs was assessed with Rome IV criteria.

Results

The mean age at study enrollment was 12.58 ± 3.79 and 9.71 ± 3.59 years in FMF and healthy control groups, respectively. Overall FGID frequency was 39.8% (n = 41) in FMF patients and 19% (n = 19) in the control group. Functional dyspepsia and irritable bowel syndrome (particularly constipation predominant subtype) rates were statistically higher in the FMF group. In detail, genotype, age at onset, symptoms, colchicine duration, and colchicine responses did not differ between FMF patients in regard to having FGIDs.

Conclusions

This study showed that children with FMF may predispose to pain predominant FGIDs. We also suggest that FGIDs should be considered in FMF patients suffering recurrent abdominal pain episodes unaccompanied by APR elevation, which can be also named as incomplete FMF attacks.
Literatur
2.
Zurück zum Zitat Park YH, Wood G, Kastner DL, Chae JJ (2016) Pyrin inflammasome activation and RhoA signaling in the autoinflammatory diseases FMF and HIDS. Nat Immunol 17:914–921CrossRefPubMedPubMedCentral Park YH, Wood G, Kastner DL, Chae JJ (2016) Pyrin inflammasome activation and RhoA signaling in the autoinflammatory diseases FMF and HIDS. Nat Immunol 17:914–921CrossRefPubMedPubMedCentral
3.
Zurück zum Zitat de Jesus AA, Canna SW, Liu Y, Goldbach-Mansky R (2015) Molecular mechanisms in genetically defined autoinflammatory diseases: disorders of amplified danger signaling. Annu Rev Immunol 33:823–874CrossRefPubMedPubMedCentral de Jesus AA, Canna SW, Liu Y, Goldbach-Mansky R (2015) Molecular mechanisms in genetically defined autoinflammatory diseases: disorders of amplified danger signaling. Annu Rev Immunol 33:823–874CrossRefPubMedPubMedCentral
4.
Zurück zum Zitat Ozen S, Demirkaya E, Erer, Livneh A, Ben-Chetrit E, Giancane G, Ozdogan H, Abu I, Gattorno M, Hawkins PN, Yuce S, Kallinich T, Bilginer Y, Kastner D, Carmona L (2016) EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis 75:644–651CrossRefPubMed Ozen S, Demirkaya E, Erer, Livneh A, Ben-Chetrit E, Giancane G, Ozdogan H, Abu I, Gattorno M, Hawkins PN, Yuce S, Kallinich T, Bilginer Y, Kastner D, Carmona L (2016) EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis 75:644–651CrossRefPubMed
5.
Zurück zum Zitat Koloski NA, Talley NJ, Boyce PM (2002) Epidemiology and health care seeking in the functional GI disorders: a population-based study. Am J Gastroenterol 97:2290–2299CrossRefPubMed Koloski NA, Talley NJ, Boyce PM (2002) Epidemiology and health care seeking in the functional GI disorders: a population-based study. Am J Gastroenterol 97:2290–2299CrossRefPubMed
6.
Zurück zum Zitat Keely S, Walker MM, Marks E, Talley NJ (2015) Immune dysregulation in the functional gastrointestinal disorders. Eur J Clin Investig 45:1350–1359CrossRef Keely S, Walker MM, Marks E, Talley NJ (2015) Immune dysregulation in the functional gastrointestinal disorders. Eur J Clin Investig 45:1350–1359CrossRef
7.
Zurück zum Zitat Kisla Ekinci RM, Balci S, Mart OO, Tumgor G, Yavuz S, Celik H, Dogruel D, Altintas DU, Yilmaz M (2018 published on 12 August 2018) Is Henoch-Schönlein purpura a susceptibility factor for functional gastrointestinal disorders in children? Rheumatol Int. https://doi.org/10.1007/s00296-018-4129-7 Kisla Ekinci RM, Balci S, Mart OO, Tumgor G, Yavuz S, Celik H, Dogruel D, Altintas DU, Yilmaz M (2018 published on 12 August 2018) Is Henoch-Schönlein purpura a susceptibility factor for functional gastrointestinal disorders in children? Rheumatol Int. https://​doi.​org/​10.​1007/​s00296-018-4129-7
8.
Zurück zum Zitat Rajindrajith S, Zeevenhooven J, Devanarayana NM, Perera BJC, Benninga MA (2018) Functional abdominal pain disorders in children. Expert Rev Gastroenterol Hepatol 12:369–390CrossRefPubMed Rajindrajith S, Zeevenhooven J, Devanarayana NM, Perera BJC, Benninga MA (2018) Functional abdominal pain disorders in children. Expert Rev Gastroenterol Hepatol 12:369–390CrossRefPubMed
9.
Zurück zum Zitat Hyams JS, Di Lorenzo C, Saps M, Perera BJC, Benninga MA (2016) Functional disorders: children and adolescents. Gastroenterology 150 e2:1456–1468CrossRef Hyams JS, Di Lorenzo C, Saps M, Perera BJC, Benninga MA (2016) Functional disorders: children and adolescents. Gastroenterology 150 e2:1456–1468CrossRef
10.
Zurück zum Zitat Barut K, Sahin S, Adrovic A, Sinoplu AB, Yucel G, Pamuk G, Aydın AK, Dasdemir S, Turanlı ET, Buyru N, Kasapcopur O (2018) Familial Mediterranean fever in childhood: a single-center experience. Rheumatol Int 38:67–74CrossRefPubMed Barut K, Sahin S, Adrovic A, Sinoplu AB, Yucel G, Pamuk G, Aydın AK, Dasdemir S, Turanlı ET, Buyru N, Kasapcopur O (2018) Familial Mediterranean fever in childhood: a single-center experience. Rheumatol Int 38:67–74CrossRefPubMed
11.
Zurück zum Zitat Tunca M, Akar S, Onen F, Ozdogan H, Kasapcopur O, Yalcinkaya F, Tutar E, Ozen S, Topaloglu R, Yilmaz E, Arici M, Bakkaloglu A, Besbas N, Akpolat T, Dinc A, Erken E, Turkish FMF Study Group (2005) Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore) 84:1–11CrossRef Tunca M, Akar S, Onen F, Ozdogan H, Kasapcopur O, Yalcinkaya F, Tutar E, Ozen S, Topaloglu R, Yilmaz E, Arici M, Bakkaloglu A, Besbas N, Akpolat T, Dinc A, Erken E, Turkish FMF Study Group (2005) Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore) 84:1–11CrossRef
12.
Zurück zum Zitat Mor A, Gal R, Livneh A (2003) Abdominal and digestive system associations of familial Mediterranean fever. Am J Gastroenterol 98:2594–2604CrossRefPubMed Mor A, Gal R, Livneh A (2003) Abdominal and digestive system associations of familial Mediterranean fever. Am J Gastroenterol 98:2594–2604CrossRefPubMed
13.
Zurück zum Zitat Asakura K, Yanai S, Nakamura S, Kawaski K, Eizuka M, Ishida K, Endo M, Sugai T, Migita K, Matsumoto T (2018) Familial Mediterranean fever mimicking Crohn disease: a case report. Medicine (Baltimore) 97:e9547CrossRef Asakura K, Yanai S, Nakamura S, Kawaski K, Eizuka M, Ishida K, Endo M, Sugai T, Migita K, Matsumoto T (2018) Familial Mediterranean fever mimicking Crohn disease: a case report. Medicine (Baltimore) 97:e9547CrossRef
14.
Zurück zum Zitat Güncan S, Bilge NŞ, Cansu DÜ, Kaşifoğlu T, Korkmaz C (2016) The role of MEFV mutations in the concurrent disorders observed in patients with familial Mediterranean fever. Eur J Rheumatol 3:118–121CrossRefPubMedPubMedCentral Güncan S, Bilge NŞ, Cansu DÜ, Kaşifoğlu T, Korkmaz C (2016) The role of MEFV mutations in the concurrent disorders observed in patients with familial Mediterranean fever. Eur J Rheumatol 3:118–121CrossRefPubMedPubMedCentral
15.
Zurück zum Zitat Özçakar ZB, Çakar N, Uncu N, Çelikel BA, Yalçinkaya F (2017) Familial Mediterranean fever-associated diseases in children. QJM 110:287–290PubMed Özçakar ZB, Çakar N, Uncu N, Çelikel BA, Yalçinkaya F (2017) Familial Mediterranean fever-associated diseases in children. QJM 110:287–290PubMed
16.
Zurück zum Zitat Fidder HH, Chowers Y, Lidar M, Sternberg M, Langevitz P, Livneh A (2002) Crohn disease in patients with familial Mediterranean fever. Medicine 81:411–416CrossRefPubMed Fidder HH, Chowers Y, Lidar M, Sternberg M, Langevitz P, Livneh A (2002) Crohn disease in patients with familial Mediterranean fever. Medicine 81:411–416CrossRefPubMed
17.
Zurück zum Zitat Papin S, Cuenin S, Agostini L, Martinon F, Werner S, Beer HD, Grütter C, Grütter M, Tschopp J (2007) The SPRY domain of pyrin, mutated in familial Mediterranean fever patients, interacts with inflammasome components and inhibits proIL-1beta processing. Cell Death Differ 14:1457–1466CrossRefPubMed Papin S, Cuenin S, Agostini L, Martinon F, Werner S, Beer HD, Grütter C, Grütter M, Tschopp J (2007) The SPRY domain of pyrin, mutated in familial Mediterranean fever patients, interacts with inflammasome components and inhibits proIL-1beta processing. Cell Death Differ 14:1457–1466CrossRefPubMed
18.
Zurück zum Zitat Yu JW, Fernandes-Alnemri T, Datta P, Wu J, Juliana C, Solorzano L, McCormick M, Zhang Z, Alnemri ES (2007) Pyrin activates the ASC pyroptosome in response to engagement by autoinflammatory PSTPIP1 mutants. Mol Cell 28:214–227CrossRefPubMedPubMedCentral Yu JW, Fernandes-Alnemri T, Datta P, Wu J, Juliana C, Solorzano L, McCormick M, Zhang Z, Alnemri ES (2007) Pyrin activates the ASC pyroptosome in response to engagement by autoinflammatory PSTPIP1 mutants. Mol Cell 28:214–227CrossRefPubMedPubMedCentral
19.
Zurück zum Zitat Chae JJ, Cho YH, Lee GS, Cheng J, Liu PP, Feigenbaum L, Katz SI, Kastner DL (2011) Gain-of-function pyrin mutations induce NLRP3 protein-independent interleukin-1β activation and severe autoinflammation in mice. Immunity 34:755–768CrossRefPubMedPubMedCentral Chae JJ, Cho YH, Lee GS, Cheng J, Liu PP, Feigenbaum L, Katz SI, Kastner DL (2011) Gain-of-function pyrin mutations induce NLRP3 protein-independent interleukin-1β activation and severe autoinflammation in mice. Immunity 34:755–768CrossRefPubMedPubMedCentral
20.
21.
Zurück zum Zitat Farmer AD, Aziz Q (2014) Mechanisms of visceral pain in health and functional gastrointestinal disorders. Scand J Pain 5:51–60CrossRefPubMed Farmer AD, Aziz Q (2014) Mechanisms of visceral pain in health and functional gastrointestinal disorders. Scand J Pain 5:51–60CrossRefPubMed
22.
Zurück zum Zitat Brik R, Litmanovich D, Berkowitz D, Shamir R, Rosenthal E, Shinawi M, Gershoni-Baruch R (2001) Incidence of familial Mediterranean fever (FMF) mutations among children of Mediterranean extraction with functional abdominal pain. J Pediatr 138:759–762CrossRefPubMed Brik R, Litmanovich D, Berkowitz D, Shamir R, Rosenthal E, Shinawi M, Gershoni-Baruch R (2001) Incidence of familial Mediterranean fever (FMF) mutations among children of Mediterranean extraction with functional abdominal pain. J Pediatr 138:759–762CrossRefPubMed
23.
Zurück zum Zitat Börekci E, Celikbilek M, Soytürk M, Akar S, Börekci H, Günaydin I (2017) Functional gastrointestinal disorders in patients with familial Mediterranean fever. Int J Rheum Dis 20:2101–2105CrossRefPubMed Börekci E, Celikbilek M, Soytürk M, Akar S, Börekci H, Günaydin I (2017) Functional gastrointestinal disorders in patients with familial Mediterranean fever. Int J Rheum Dis 20:2101–2105CrossRefPubMed
24.
Zurück zum Zitat Kucuk A, Biyik M, Solak Y, Ucar R, Polat I, Pektas F, Gungor G, Gaipov A, Bagcaci S, Ataseven H, Tunc R (2015) Irritable bowel syndrome prevalence in patients with familial Mediterranean fever. Acta Medica Mediterranea 31:841–846 Kucuk A, Biyik M, Solak Y, Ucar R, Polat I, Pektas F, Gungor G, Gaipov A, Bagcaci S, Ataseven H, Tunc R (2015) Irritable bowel syndrome prevalence in patients with familial Mediterranean fever. Acta Medica Mediterranea 31:841–846
25.
Zurück zum Zitat Ozçakar ZB, Yalçinkaya F, Yüksel S, Acar B, Gökmen D, Ekim M (2006) Possible effect of subclinical inflammation on daily life in familial Mediterranean fever. Clin Rheumatol 25(2):149–152CrossRefPubMed Ozçakar ZB, Yalçinkaya F, Yüksel S, Acar B, Gökmen D, Ekim M (2006) Possible effect of subclinical inflammation on daily life in familial Mediterranean fever. Clin Rheumatol 25(2):149–152CrossRefPubMed
Metadaten
Titel
Frequency of functional gastrointestinal disorders in children with familial Mediterranean fever
verfasst von
Rabia Miray Kisla Ekinci
Sibel Balcı
Eray Akay
Gokhan Tumgor
Dilek Dogruel
Derya Ufuk Altintas
Mustafa Yilmaz
Publikationsdatum
31.01.2019
Verlag
Springer London
Erschienen in
Clinical Rheumatology / Ausgabe 3/2019
Print ISSN: 0770-3198
Elektronische ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-019-04452-1

Weitere Artikel der Ausgabe 3/2019

Clinical Rheumatology 3/2019 Zur Ausgabe

Leitlinien kompakt für die Innere Medizin

Mit medbee Pocketcards sicher entscheiden.

Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Update Innere Medizin

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.