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Erschienen in: Zeitschrift für Rheumatologie 5/2019

14.05.2019 | Rheumatologische Erkrankungen in der Hausarztpraxis | CME

Systemische Sklerose – klinisches Bild, Diagnostik und Therapie

verfasst von: Dr. med. M. O. Becker, O. Distler, B. Maurer

Erschienen in: Zeitschrift für Rheumatologie | Ausgabe 5/2019

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Zusammenfassung

Die systemische Sklerose ist eine seltene rheumatische Erkrankung, die durch Haut- und Organfibrose, vaskuläre Veränderungen und das Auftreten spezifischer Autoantikörper charakterisiert ist. Sie zeigt eine hohe Morbidität und Mortalität, weist dabei aber in ihrem Verlauf und den Manifestationen eine beträchtliche Heterogenität auf. Dies macht eine umfassende Diagnostik zur Diagnosestellung und bei den Verlaufsuntersuchungen notwendig, da auch die Therapie den jeweiligen Krankheitsmanifestationen angepasst werden muss. Obwohl spezifische Therapien für gastrointestinale, pulmonale oder auch vaskuläre Komplikationen existieren, sprechen die Patienten meistens nur teilweise an und neue Therapieansätze sind notwendig.
Literatur
1.
Zurück zum Zitat Gabrielli A, Avvedimento EV, Scleroderma KT (2009) N Engl J Med 360(19):1989–2003 PubMed Gabrielli A, Avvedimento EV, Scleroderma KT (2009) N Engl J Med 360(19):1989–2003 PubMed
2.
Zurück zum Zitat Allanore Y, Simms R, Distler O, Trojanowska M, Pope J, Denton CP et al (2015) Systemic sclerosis. Nat Rev Dis Primers 1:15002 PubMed Allanore Y, Simms R, Distler O, Trojanowska M, Pope J, Denton CP et al (2015) Systemic sclerosis. Nat Rev Dis Primers 1:15002 PubMed
3.
Zurück zum Zitat Katsumoto TR, Whitfield ML, Connolly MK (2011) The pathogenesis of systemic sclerosis. Annu Rev Pathol 6:509–537 PubMed Katsumoto TR, Whitfield ML, Connolly MK (2011) The pathogenesis of systemic sclerosis. Annu Rev Pathol 6:509–537 PubMed
4.
Zurück zum Zitat Aringer M, Muller-Ladner U, Burkhardt H, Distler JH, Distler O, Graninger WB et al (2015) Common German language nomenclature for systemic sclerosis. Z Rheumatol 74(2):100–103 PubMed Aringer M, Muller-Ladner U, Burkhardt H, Distler JH, Distler O, Graninger WB et al (2015) Common German language nomenclature for systemic sclerosis. Z Rheumatol 74(2):100–103 PubMed
5.
Zurück zum Zitat Andreasson K, Saxne T, Bergknut C, Hesselstrand R, Englund M (2014) Prevalence and incidence of systemic sclerosis in southern Sweden: population-based data with case ascertainment using the 1980 ARA criteria and the proposed ACR-EULAR classification criteria. Ann Rheum Dis 73(10):1788–1792 PubMed Andreasson K, Saxne T, Bergknut C, Hesselstrand R, Englund M (2014) Prevalence and incidence of systemic sclerosis in southern Sweden: population-based data with case ascertainment using the 1980 ARA criteria and the proposed ACR-EULAR classification criteria. Ann Rheum Dis 73(10):1788–1792 PubMed
6.
Zurück zum Zitat Chifflot H, Fautrel B, Sordet C, Chatelus E, Sibilia J (2008) Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum 37(4):223–235 PubMed Chifflot H, Fautrel B, Sordet C, Chatelus E, Sibilia J (2008) Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum 37(4):223–235 PubMed
7.
Zurück zum Zitat Mayes MD (2003) Scleroderma epidemiology. Rheum Dis Clin North Am 29(2):239–254 PubMed Mayes MD (2003) Scleroderma epidemiology. Rheum Dis Clin North Am 29(2):239–254 PubMed
8.
Zurück zum Zitat Meier FM, Frommer KW, Dinser R, Walker UA, Czirjak L, Denton CP et al (2012) Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis 71(8):1355–1360 PubMed Meier FM, Frommer KW, Dinser R, Walker UA, Czirjak L, Denton CP et al (2012) Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis 71(8):1355–1360 PubMed
9.
Zurück zum Zitat Zulian F (2017) Scleroderma in children. Best Pract Res Clin Rheumatol 31(4):576–595 PubMed Zulian F (2017) Scleroderma in children. Best Pract Res Clin Rheumatol 31(4):576–595 PubMed
10.
Zurück zum Zitat Elhai M, Meune C, Avouac J, Kahan A, Allanore Y (2012) Trends in mortality in patients with systemic sclerosis over 40 years: a systematic review and meta-analysis of cohort studies. Rheumatology 51(6):1017–1026 PubMed Elhai M, Meune C, Avouac J, Kahan A, Allanore Y (2012) Trends in mortality in patients with systemic sclerosis over 40 years: a systematic review and meta-analysis of cohort studies. Rheumatology 51(6):1017–1026 PubMed
11.
Zurück zum Zitat Ferri C, Valentini G, Cozzi F, Sebastiani M, Michelassi C, La Montagna G et al (2002) Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Medicine (Baltimore) 81(2):139–153 Ferri C, Valentini G, Cozzi F, Sebastiani M, Michelassi C, La Montagna G et al (2002) Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Medicine (Baltimore) 81(2):139–153
12.
Zurück zum Zitat Steen VD, Medsger TA (2007) Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 66(7):940–944 PubMedPubMedCentral Steen VD, Medsger TA (2007) Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 66(7):940–944 PubMedPubMedCentral
13.
Zurück zum Zitat Komocsi A, Vorobcsuk A, Faludi R, Pinter T, Lenkey Z, Kolto G et al (2012) The impact of cardiopulmonary manifestations on the mortality of SSc: a systematic review and meta-analysis of observational studies. Rheumatology 51(6):1027–1036 PubMed Komocsi A, Vorobcsuk A, Faludi R, Pinter T, Lenkey Z, Kolto G et al (2012) The impact of cardiopulmonary manifestations on the mortality of SSc: a systematic review and meta-analysis of observational studies. Rheumatology 51(6):1027–1036 PubMed
14.
Zurück zum Zitat Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE et al (2010) Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 69(10):1809–1815 PubMed Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE et al (2010) Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 69(10):1809–1815 PubMed
15.
Zurück zum Zitat Elhai M, Meune C, Boubaya M, Avouac J, Hachulla E, Balbir-Gurman A et al (2017) Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis 76(11):1897–1905 PubMed Elhai M, Meune C, Boubaya M, Avouac J, Hachulla E, Balbir-Gurman A et al (2017) Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis 76(11):1897–1905 PubMed
16.
Zurück zum Zitat Launay D, Montani D, Hassoun PM, Cottin V, Le Pavec J, Clerson P et al (2018) Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis. PLoS ONE 13(5):e197112 PubMedPubMedCentral Launay D, Montani D, Hassoun PM, Cottin V, Le Pavec J, Clerson P et al (2018) Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis. PLoS ONE 13(5):e197112 PubMedPubMedCentral
17.
Zurück zum Zitat Koh ET, Lee P, Gladman DD, Abu-Shakra M (1996) Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 35(10):989–993 PubMed Koh ET, Lee P, Gladman DD, Abu-Shakra M (1996) Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 35(10):989–993 PubMed
18.
Zurück zum Zitat Humbert M, Yaici A, de Groote P, Montani D, Sitbon O, Launay D et al (2011) Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum 63(11):3522–3530 PubMed Humbert M, Yaici A, de Groote P, Montani D, Sitbon O, Launay D et al (2011) Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum 63(11):3522–3530 PubMed
19.
Zurück zum Zitat Jordan S, Chung J, Distler O (2013) Preclinical and translational research to discover potentially effective antifibrotic therapies in systemic sclerosis. Curr Opin Rheumatol 25(6):679–685 PubMed Jordan S, Chung J, Distler O (2013) Preclinical and translational research to discover potentially effective antifibrotic therapies in systemic sclerosis. Curr Opin Rheumatol 25(6):679–685 PubMed
20.
Zurück zum Zitat Marangoni RG, Varga J, Tourtellotte WG (2016) Animal models of scleroderma: recent progress. Curr Opin Rheumatol 28(6):561–570 PubMed Marangoni RG, Varga J, Tourtellotte WG (2016) Animal models of scleroderma: recent progress. Curr Opin Rheumatol 28(6):561–570 PubMed
21.
Zurück zum Zitat Airo P, Ceribelli A, Cavazzana I, Taraborelli M, Zingarelli S, Franceschini F (2011) Malignancies in Italian patients with systemic sclerosis positive for anti-RNA polymerase III antibodies. J Rheumatol 38(7):1329–1334 PubMed Airo P, Ceribelli A, Cavazzana I, Taraborelli M, Zingarelli S, Franceschini F (2011) Malignancies in Italian patients with systemic sclerosis positive for anti-RNA polymerase III antibodies. J Rheumatol 38(7):1329–1334 PubMed
22.
Zurück zum Zitat Moinzadeh P, Fonseca C, Hellmich M, Shah AA, Chighizola C, Denton CP et al (2014) Association of anti-RNA polymerase III autoantibodies and cancer in scleroderma. Arthritis Res Ther 16(1):R53 PubMedPubMedCentral Moinzadeh P, Fonseca C, Hellmich M, Shah AA, Chighizola C, Denton CP et al (2014) Association of anti-RNA polymerase III autoantibodies and cancer in scleroderma. Arthritis Res Ther 16(1):R53 PubMedPubMedCentral
23.
Zurück zum Zitat Patterson KA, Roberts-Thomson PJ, Lester S, Tan JA, Hakendorf P, Rischmueller M et al (2015) Interpretation of an extended autoantibody profile in a well-characterized Australian systemic sclerosis (Scleroderma) cohort using principal components analysis. Arthritis Rheumatol 67(12):3234–3244 PubMed Patterson KA, Roberts-Thomson PJ, Lester S, Tan JA, Hakendorf P, Rischmueller M et al (2015) Interpretation of an extended autoantibody profile in a well-characterized Australian systemic sclerosis (Scleroderma) cohort using principal components analysis. Arthritis Rheumatol 67(12):3234–3244 PubMed
24.
Zurück zum Zitat Saigusa R, Asano Y, Nakamura K, Miura S, Ichimura Y, Takahashi T et al (2015) Association of anti-RNA polymerase III antibody and malignancy in Japanese patients with systemic sclerosis. J Dermatol 42(5):524–527 PubMed Saigusa R, Asano Y, Nakamura K, Miura S, Ichimura Y, Takahashi T et al (2015) Association of anti-RNA polymerase III antibody and malignancy in Japanese patients with systemic sclerosis. J Dermatol 42(5):524–527 PubMed
25.
Zurück zum Zitat Shah AA, Rosen A, Hummers L, Wigley F, Casciola-Rosen L (2010) Close temporal relationship between onset of cancer and scleroderma in patients with RNA polymerase I/III antibodies. Arthritis Rheum 62(9):2787–2795 PubMedPubMedCentral Shah AA, Rosen A, Hummers L, Wigley F, Casciola-Rosen L (2010) Close temporal relationship between onset of cancer and scleroderma in patients with RNA polymerase I/III antibodies. Arthritis Rheum 62(9):2787–2795 PubMedPubMedCentral
26.
Zurück zum Zitat Joseph CG, Darrah E, Shah AA, Skora AD, Casciola-Rosen LA, Wigley FM et al (2014) Association of the autoimmune disease scleroderma with an immunologic response to cancer. Science 343(6167):152–157 PubMed Joseph CG, Darrah E, Shah AA, Skora AD, Casciola-Rosen LA, Wigley FM et al (2014) Association of the autoimmune disease scleroderma with an immunologic response to cancer. Science 343(6167):152–157 PubMed
27.
Zurück zum Zitat Frerix M, Meier FM, Muller-Ladner U (2013) The road to early diagnosis of systemic sclerosis : the evolution of diagnostic and classification criteria in the past decades. Z Rheumatol 72(10):954–959 PubMed Frerix M, Meier FM, Muller-Ladner U (2013) The road to early diagnosis of systemic sclerosis : the evolution of diagnostic and classification criteria in the past decades. Z Rheumatol 72(10):954–959 PubMed
29.
Zurück zum Zitat LeRoy EC, Medsger TA Jr. (2001) Criteria for the classification of early systemic sclerosis. J Rheumatol 28(7):1573–1576 PubMed LeRoy EC, Medsger TA Jr. (2001) Criteria for the classification of early systemic sclerosis. J Rheumatol 28(7):1573–1576 PubMed
30.
Zurück zum Zitat Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 23(5):581–590 Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 23(5):581–590
31.
Zurück zum Zitat Jordan S, Maurer B, Toniolo M, Michel B, Distler O (2015) Performance of the new ACR/EULAR classification criteria for systemic sclerosis in clinical practice. Rheumatology 54(8):1454–1458 PubMed Jordan S, Maurer B, Toniolo M, Michel B, Distler O (2015) Performance of the new ACR/EULAR classification criteria for systemic sclerosis in clinical practice. Rheumatology 54(8):1454–1458 PubMed
32.
Zurück zum Zitat van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A et al (2013) 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum 65(11):2737–2747 PubMedPubMedCentral van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A et al (2013) 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum 65(11):2737–2747 PubMedPubMedCentral
33.
Zurück zum Zitat Alhajeri H, Hudson M, Fritzler M, Pope J, Tatibouet S, Markland J et al (2015) 2013 American College of Rheumatology/European League against rheumatism classification criteria for systemic sclerosis outperform the 1980 criteria: data from the Canadian Scleroderma Research Group. Arthritis Care Res (Hoboken) 67(4):582–587 Alhajeri H, Hudson M, Fritzler M, Pope J, Tatibouet S, Markland J et al (2015) 2013 American College of Rheumatology/European League against rheumatism classification criteria for systemic sclerosis outperform the 1980 criteria: data from the Canadian Scleroderma Research Group. Arthritis Care Res (Hoboken) 67(4):582–587
34.
Zurück zum Zitat Saez-Comet L, Simeon-Aznar CP, Perez-Conesa M, Vallejo-Rodriguez C, Tolosa-Vilella C, Iniesta-Arandia N et al (2015) Applying the ACR/EULAR Systemic Sclerosis Classification Criteria to the Spanish Scleroderma Registry Cohort. J Rheumatol 42(12):2327–2331 PubMed Saez-Comet L, Simeon-Aznar CP, Perez-Conesa M, Vallejo-Rodriguez C, Tolosa-Vilella C, Iniesta-Arandia N et al (2015) Applying the ACR/EULAR Systemic Sclerosis Classification Criteria to the Spanish Scleroderma Registry Cohort. J Rheumatol 42(12):2327–2331 PubMed
35.
Zurück zum Zitat Walker UA, Knoss P, Jakobs M, Krenn V (2009) Scleroderma and fibrosing diseases. Z Rheumatol 68(4):312–319 PubMed Walker UA, Knoss P, Jakobs M, Krenn V (2009) Scleroderma and fibrosing diseases. Z Rheumatol 68(4):312–319 PubMed
36.
Zurück zum Zitat Nashel J, Steen V (2012) Scleroderma mimics. Curr Rheumatol Rep 14(1):39–46 PubMed Nashel J, Steen V (2012) Scleroderma mimics. Curr Rheumatol Rep 14(1):39–46 PubMed
37.
Zurück zum Zitat Constantin T, Foeldvari I, Pain CE et al (2018) Development of minimum standards of care for juvenile localized scleroderma. Eur J Pediatr 177:961–977 PubMed Constantin T, Foeldvari I, Pain CE et al (2018) Development of minimum standards of care for juvenile localized scleroderma. Eur J Pediatr 177:961–977 PubMed
38.
Zurück zum Zitat Minier T, Guiducci S, Bellando-Randone S, Bruni C, Lepri G, Czirjak L et al (2014) Preliminary analysis of the very early diagnosis of systemic sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis. Ann Rheum Dis 73(12):2087–2093 PubMed Minier T, Guiducci S, Bellando-Randone S, Bruni C, Lepri G, Czirjak L et al (2014) Preliminary analysis of the very early diagnosis of systemic sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis. Ann Rheum Dis 73(12):2087–2093 PubMed
39.
Zurück zum Zitat Lepri G, Guiducci S, Bellando-Randone S, Giani I, Bruni C, Blagojevic J et al (2015) Evidence for oesophageal and anorectal involvement in very early systemic sclerosis (VEDOSS): report from a single VEDOSS/EUSTAR centre. Ann Rheum Dis 74(1):124–128 PubMed Lepri G, Guiducci S, Bellando-Randone S, Giani I, Bruni C, Blagojevic J et al (2015) Evidence for oesophageal and anorectal involvement in very early systemic sclerosis (VEDOSS): report from a single VEDOSS/EUSTAR centre. Ann Rheum Dis 74(1):124–128 PubMed
40.
Zurück zum Zitat Bruni C, Guiducci S, Bellando-Randone S, Lepri G, Braschi F, Fiori G et al (2015) Digital ulcers as a sentinel sign for early internal organ involvement in very early systemic sclerosis. Rheumatology 54(1):72–76 PubMed Bruni C, Guiducci S, Bellando-Randone S, Lepri G, Braschi F, Fiori G et al (2015) Digital ulcers as a sentinel sign for early internal organ involvement in very early systemic sclerosis. Rheumatology 54(1):72–76 PubMed
42.
Zurück zum Zitat LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr. et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15(2):202–205 PubMed LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr. et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15(2):202–205 PubMed
43.
Zurück zum Zitat Barnett AJ, Miller MH, Littlejohn GO (1988) A survival study of patients with scleroderma diagnosed over 30 years (1953–1983): the value of a simple cutaneous classification in the early stages of the disease. J Rheumatol 15(2):276–283 PubMed Barnett AJ, Miller MH, Littlejohn GO (1988) A survival study of patients with scleroderma diagnosed over 30 years (1953–1983): the value of a simple cutaneous classification in the early stages of the disease. J Rheumatol 15(2):276–283 PubMed
44.
Zurück zum Zitat Scussel-Lonzetti L, Joyal F, Raynauld JP, Roussin A, Rich E, Goulet JR et al (2002) Predicting mortality in systemic sclerosis: analysis of a cohort of 309 French Canadian patients with emphasis on features at diagnosis as predictive factors for survival. Medicine (Baltimore) 81(2):154–167 Scussel-Lonzetti L, Joyal F, Raynauld JP, Roussin A, Rich E, Goulet JR et al (2002) Predicting mortality in systemic sclerosis: analysis of a cohort of 309 French Canadian patients with emphasis on features at diagnosis as predictive factors for survival. Medicine (Baltimore) 81(2):154–167
45.
Zurück zum Zitat Al-Dhaher FF, Pope JE, Ouimet JM (2010) Determinants of morbidity and mortality of systemic sclerosis in Canada. Semin Arthritis Rheum 39(4):269–277 PubMed Al-Dhaher FF, Pope JE, Ouimet JM (2010) Determinants of morbidity and mortality of systemic sclerosis in Canada. Semin Arthritis Rheum 39(4):269–277 PubMed
46.
Zurück zum Zitat Hachulla E, Carpentier P, Gressin V, Diot E, Allanore Y, Sibilia J et al (2009) Risk factors for death and the 3‑year survival of patients with systemic sclerosis: the French ItinerAIR-Sclerodermie study. Rheumatology 48(3):304–308 PubMedPubMedCentral Hachulla E, Carpentier P, Gressin V, Diot E, Allanore Y, Sibilia J et al (2009) Risk factors for death and the 3‑year survival of patients with systemic sclerosis: the French ItinerAIR-Sclerodermie study. Rheumatology 48(3):304–308 PubMedPubMedCentral
47.
Zurück zum Zitat Domsic RT, Rodriguez-Reyna T, Lucas M, Fertig N, Medsger TA Jr. (2011) Skin thickness progression rate: a predictor of mortality and early internal organ involvement in diffuse scleroderma. Ann Rheum Dis 70(1):104–109 PubMed Domsic RT, Rodriguez-Reyna T, Lucas M, Fertig N, Medsger TA Jr. (2011) Skin thickness progression rate: a predictor of mortality and early internal organ involvement in diffuse scleroderma. Ann Rheum Dis 70(1):104–109 PubMed
48.
Zurück zum Zitat Domsic RT, Nihtyanova SI, Wisniewski SR, Fine MJ, Lucas M, Kwoh CK et al (2014) Derivation and validation of a prediction rule for two-year mortality in early diffuse cutaneous systemic sclerosis. Arthritis Rheumatol 66(6):1616–1624 PubMedPubMedCentral Domsic RT, Nihtyanova SI, Wisniewski SR, Fine MJ, Lucas M, Kwoh CK et al (2014) Derivation and validation of a prediction rule for two-year mortality in early diffuse cutaneous systemic sclerosis. Arthritis Rheumatol 66(6):1616–1624 PubMedPubMedCentral
49.
Zurück zum Zitat Shand L, Lunt M, Nihtyanova S, Hoseini M, Silman A, Black CM et al (2007) Relationship between change in skin score and disease outcome in diffuse cutaneous systemic sclerosis: application of a latent linear trajectory model. Arthritis Rheum 56(7):2422–2431 PubMed Shand L, Lunt M, Nihtyanova S, Hoseini M, Silman A, Black CM et al (2007) Relationship between change in skin score and disease outcome in diffuse cutaneous systemic sclerosis: application of a latent linear trajectory model. Arthritis Rheum 56(7):2422–2431 PubMed
50.
Zurück zum Zitat Diab S, Dostrovsky N, Hudson M, Tatibouet S, Fritzler MJ, Baron M et al (2014) Systemic sclerosis sine scleroderma: a multicenter study of 1417 subjects. J Rheumatol 41(11):2179–2185 PubMed Diab S, Dostrovsky N, Hudson M, Tatibouet S, Fritzler MJ, Baron M et al (2014) Systemic sclerosis sine scleroderma: a multicenter study of 1417 subjects. J Rheumatol 41(11):2179–2185 PubMed
51.
Zurück zum Zitat Hanitsch LG, Burmester GR, Witt C, Hunzelmann N, Genth E, Krieg T et al (2009) Skin sclerosis is only of limited value to identify SSc patients with severe manifestations—an analysis of a distinct patient subgroup of the German Systemic Sclerosis Network (DNSS) Register. Rheumatology 48(1):70–73 PubMed Hanitsch LG, Burmester GR, Witt C, Hunzelmann N, Genth E, Krieg T et al (2009) Skin sclerosis is only of limited value to identify SSc patients with severe manifestations—an analysis of a distinct patient subgroup of the German Systemic Sclerosis Network (DNSS) Register. Rheumatology 48(1):70–73 PubMed
52.
Zurück zum Zitat Clements P, Lachenbruch P, Siebold J, White B, Weiner S, Martin R et al (1995) Inter and intraobserver variability of total skin thickness score (modified Rodnan TSS) in systemic sclerosis. J Rheumatol 22(7):1281–1285 PubMed Clements P, Lachenbruch P, Siebold J, White B, Weiner S, Martin R et al (1995) Inter and intraobserver variability of total skin thickness score (modified Rodnan TSS) in systemic sclerosis. J Rheumatol 22(7):1281–1285 PubMed
53.
Zurück zum Zitat Furst DE, Clements PJ, Steen VD, Medsger TA Jr., Masi AT, D’Angelo WA et al (1998) The modified Rodnan skin score is an accurate reflection of skin biopsy thickness in systemic sclerosis. J Rheumatol 25(1):84–88 PubMed Furst DE, Clements PJ, Steen VD, Medsger TA Jr., Masi AT, D’Angelo WA et al (1998) The modified Rodnan skin score is an accurate reflection of skin biopsy thickness in systemic sclerosis. J Rheumatol 25(1):84–88 PubMed
54.
Zurück zum Zitat Jaeger VK, Distler O, Maurer B, Czirjak L, Lorand V, Valentini G et al (2018) Functional disability and its predictors in systemic sclerosis: a study from the DeSScipher project within the EUSTAR group. Rheumatology 57(3):441–450 PubMed Jaeger VK, Distler O, Maurer B, Czirjak L, Lorand V, Valentini G et al (2018) Functional disability and its predictors in systemic sclerosis: a study from the DeSScipher project within the EUSTAR group. Rheumatology 57(3):441–450 PubMed
55.
Zurück zum Zitat Brand M, Hollaender R, Rosenberg D, Scott M, Hunsche E, Tyndall A et al (2015) An observational cohort study of patients with newly diagnosed digital ulcer disease secondary to systemic sclerosis registered in the EUSTAR database. Clin Exp Rheumatol 33(4 Suppl 91):S47–S54 PubMed Brand M, Hollaender R, Rosenberg D, Scott M, Hunsche E, Tyndall A et al (2015) An observational cohort study of patients with newly diagnosed digital ulcer disease secondary to systemic sclerosis registered in the EUSTAR database. Clin Exp Rheumatol 33(4 Suppl 91):S47–S54 PubMed
56.
Zurück zum Zitat Geyer M, Vasile M, Hermann W (2014) Nailfold capillaroscopy. Z Rheumatol 73(2):149–160 (quiz 61–2) PubMed Geyer M, Vasile M, Hermann W (2014) Nailfold capillaroscopy. Z Rheumatol 73(2):149–160 (quiz 61–2) PubMed
57.
Zurück zum Zitat Sander O, Sunderkotter C, Kotter I, Wagner I, Becker M, Herrgott I et al (2010) Capillaroscopy. Procedure and nomenclature. Z Rheumatol 69(3):253–262 PubMed Sander O, Sunderkotter C, Kotter I, Wagner I, Becker M, Herrgott I et al (2010) Capillaroscopy. Procedure and nomenclature. Z Rheumatol 69(3):253–262 PubMed
58.
Zurück zum Zitat Avouac J, Airo P, Meune C, Beretta L, Dieude P, Caramaschi P et al (2010) Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. J Rheumatol 37(11):2290–2298 PubMed Avouac J, Airo P, Meune C, Beretta L, Dieude P, Caramaschi P et al (2010) Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. J Rheumatol 37(11):2290–2298 PubMed
59.
Zurück zum Zitat Grund D, Siegert E (2016) Pulmonary fibrosis in rheumatic diseases. Z Rheumatol 75(8):795–808 PubMed Grund D, Siegert E (2016) Pulmonary fibrosis in rheumatic diseases. Z Rheumatol 75(8):795–808 PubMed
60.
Zurück zum Zitat Wells AU, Denton CP (2014) Interstitial lung disease in connective tissue disease—mechanisms and management. Nat Rev Rheumatol 10(12):728–739 PubMed Wells AU, Denton CP (2014) Interstitial lung disease in connective tissue disease—mechanisms and management. Nat Rev Rheumatol 10(12):728–739 PubMed
61.
Zurück zum Zitat Goh NS, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM et al (2008) Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med 177(11):1248–1254 PubMed Goh NS, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM et al (2008) Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med 177(11):1248–1254 PubMed
62.
Zurück zum Zitat Goh NS, Veeraraghavan S, Desai SR, Cramer D, Hansell DM, Denton CP et al (2007) Bronchoalveolar lavage cellular profiles in patients with systemic sclerosis-associated interstitial lung disease are not predictive of disease progression. Arthritis Rheum 56(6):2005–2012 PubMed Goh NS, Veeraraghavan S, Desai SR, Cramer D, Hansell DM, Denton CP et al (2007) Bronchoalveolar lavage cellular profiles in patients with systemic sclerosis-associated interstitial lung disease are not predictive of disease progression. Arthritis Rheum 56(6):2005–2012 PubMed
63.
Zurück zum Zitat Bouros D, Wells AU, Nicholson AG, Colby TV, Polychronopoulos V, Pantelidis P et al (2002) Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 165(12):1581–1586 PubMed Bouros D, Wells AU, Nicholson AG, Colby TV, Polychronopoulos V, Pantelidis P et al (2002) Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 165(12):1581–1586 PubMed
65.
Zurück zum Zitat Frech TM, Mar D (2018) Gastrointestinal and hepatic disease in systemic sclerosis. Rheum Dis Clin North Am 44(1):15–28 PubMedPubMedCentral Frech TM, Mar D (2018) Gastrointestinal and hepatic disease in systemic sclerosis. Rheum Dis Clin North Am 44(1):15–28 PubMedPubMedCentral
66.
Zurück zum Zitat Kumar S, Singh J, Rattan S, DiMarino AJ, Cohen S, Jimenez SA (2017) Review article: pathogenesis and clinical manifestations of gastrointestinal involvement in systemic sclerosis. Aliment Pharmacol Ther 45(7):883–898 PubMedPubMedCentral Kumar S, Singh J, Rattan S, DiMarino AJ, Cohen S, Jimenez SA (2017) Review article: pathogenesis and clinical manifestations of gastrointestinal involvement in systemic sclerosis. Aliment Pharmacol Ther 45(7):883–898 PubMedPubMedCentral
67.
Zurück zum Zitat Forbes A, Marie I (2009) Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology 48(Suppl 3):iii36–iii39 PubMed Forbes A, Marie I (2009) Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology 48(Suppl 3):iii36–iii39 PubMed
68.
Zurück zum Zitat Pope JE (2002) Scleroderma overlap syndromes. Curr Opin Rheumatol 14(6):704–710 PubMed Pope JE (2002) Scleroderma overlap syndromes. Curr Opin Rheumatol 14(6):704–710 PubMed
69.
Zurück zum Zitat Avouac J, Walker U, Tyndall A, Kahan A, Matucci-Cerinic M, Allanore Y et al (2010) Characteristics of joint involvement and relationships with systemic inflammation in systemic sclerosis: results from the EULAR Scleroderma Trial and Research Group (EUSTAR) database. J Rheumatol 37(7):1488–1501 PubMed Avouac J, Walker U, Tyndall A, Kahan A, Matucci-Cerinic M, Allanore Y et al (2010) Characteristics of joint involvement and relationships with systemic inflammation in systemic sclerosis: results from the EULAR Scleroderma Trial and Research Group (EUSTAR) database. J Rheumatol 37(7):1488–1501 PubMed
70.
Zurück zum Zitat Avouac J, Walker UA, Hachulla E, Riemekasten G, Cuomo G, Carreira PE et al (2016) Joint and tendon involvement predict disease progression in systemic sclerosis: a EUSTAR prospective study. Ann Rheum Dis 75(1):103–109 PubMed Avouac J, Walker UA, Hachulla E, Riemekasten G, Cuomo G, Carreira PE et al (2016) Joint and tendon involvement predict disease progression in systemic sclerosis: a EUSTAR prospective study. Ann Rheum Dis 75(1):103–109 PubMed
71.
Zurück zum Zitat Steen VD, Medsger TA Jr. (1997) The palpable tendon friction rub: an important physical examination finding in patients with systemic sclerosis. Arthritis Rheum 40(6):1146–1151 PubMed Steen VD, Medsger TA Jr. (1997) The palpable tendon friction rub: an important physical examination finding in patients with systemic sclerosis. Arthritis Rheum 40(6):1146–1151 PubMed
72.
Zurück zum Zitat Cuomo G, Zappia M, Iudici M, Abignano G, Rotondo A, Valentini G (2012) The origin of tendon friction rubs in patients with systemic sclerosis: a sonographic explanation. Arthritis Rheum 64(4):1291–1293 PubMed Cuomo G, Zappia M, Iudici M, Abignano G, Rotondo A, Valentini G (2012) The origin of tendon friction rubs in patients with systemic sclerosis: a sonographic explanation. Arthritis Rheum 64(4):1291–1293 PubMed
73.
Zurück zum Zitat Pope JE (2003) Musculoskeletal involvement in scleroderma. Rheum Dis Clin North Am 29(2):391–408 PubMed Pope JE (2003) Musculoskeletal involvement in scleroderma. Rheum Dis Clin North Am 29(2):391–408 PubMed
74.
Zurück zum Zitat Penn H, Howie AJ, Kingdon EJ, Bunn CC, Stratton RJ, Black CM et al (2007) Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM 100(8):485–494 PubMed Penn H, Howie AJ, Kingdon EJ, Bunn CC, Stratton RJ, Black CM et al (2007) Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM 100(8):485–494 PubMed
75.
Zurück zum Zitat Koenig M, Joyal F, Fritzler MJ, Roussin A, Abrahamowicz M, Boire G et al (2008) Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud’s phenomenon to systemic sclerosis: a twenty-year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosis. Arthritis Rheum 58(12):3902–3912 PubMed Koenig M, Joyal F, Fritzler MJ, Roussin A, Abrahamowicz M, Boire G et al (2008) Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud’s phenomenon to systemic sclerosis: a twenty-year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosis. Arthritis Rheum 58(12):3902–3912 PubMed
77.
Zurück zum Zitat Hunzelmann N, Genth E, Krieg T, Meurer M, Melchers I, Moinzadeh P et al (2008) Organ-specific diagnosis in patients with systemic sclerosis: recommendations of the German Network for Systemic Sclerosis (DNSS). Z Rheumatol 67(4):334–336, 7–40 PubMed Hunzelmann N, Genth E, Krieg T, Meurer M, Melchers I, Moinzadeh P et al (2008) Organ-specific diagnosis in patients with systemic sclerosis: recommendations of the German Network for Systemic Sclerosis (DNSS). Z Rheumatol 67(4):334–336, 7–40 PubMed
78.
Zurück zum Zitat Galluccio F, Walker UA, Nihtyanova S, Moinzadeh P, Hunzelmann N, Krieg T et al (2011) Registries in systemic sclerosis: a worldwide experience. Rheumatology 50(1):60–68 PubMed Galluccio F, Walker UA, Nihtyanova S, Moinzadeh P, Hunzelmann N, Krieg T et al (2011) Registries in systemic sclerosis: a worldwide experience. Rheumatology 50(1):60–68 PubMed
79.
Zurück zum Zitat Suliman et al (2015) Arthritis Rheumatol 67(12):3256–3261 PubMed Suliman et al (2015) Arthritis Rheumatol 67(12):3256–3261 PubMed
80.
81.
84.
Zurück zum Zitat Becker MO (2016) Systemic sclerosis : what is currently available for treatment? Internist (Berl) 57(12):1155–1163 Becker MO (2016) Systemic sclerosis : what is currently available for treatment? Internist (Berl) 57(12):1155–1163
85.
Zurück zum Zitat Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y et al (2017) Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis 76(8):1327–1339 PubMed Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y et al (2017) Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis 76(8):1327–1339 PubMed
86.
Zurück zum Zitat Fernandez-Codina A, Walker KM, Pope JE, Scleroderma Algorithm G (2018) Treatment algorithms for systemic sclerosis according to experts. Arthritis Rheumatol 70(11):1820–1828 PubMed Fernandez-Codina A, Walker KM, Pope JE, Scleroderma Algorithm G (2018) Treatment algorithms for systemic sclerosis according to experts. Arthritis Rheumatol 70(11):1820–1828 PubMed
88.
Zurück zum Zitat Sitbon O, Channick R, Chin KM, Frey A, Gaine S, Galie N et al (2015) Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 373(26):2522–2533 PubMed Sitbon O, Channick R, Chin KM, Frey A, Gaine S, Galie N et al (2015) Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 373(26):2522–2533 PubMed
89.
Zurück zum Zitat Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A et al (2015) 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 46(4):903–975 PubMed Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A et al (2015) 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 46(4):903–975 PubMed
90.
Zurück zum Zitat Johnson SR, Brode SK, Mielniczuk LM, Granton JT (2012) Dual therapy in IPAH and SSc-PAH. A qualitative systematic review. Respir Med 106(5):730–739 PubMed Johnson SR, Brode SK, Mielniczuk LM, Granton JT (2012) Dual therapy in IPAH and SSc-PAH. A qualitative systematic review. Respir Med 106(5):730–739 PubMed
91.
Zurück zum Zitat Tashkin DP, Roth MD, Clements PJ, Furst DE, Khanna D, Kleerup EC et al (2016) Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med 4(9):708–719 PubMedPubMedCentral Tashkin DP, Roth MD, Clements PJ, Furst DE, Khanna D, Kleerup EC et al (2016) Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med 4(9):708–719 PubMedPubMedCentral
92.
Zurück zum Zitat Owen C, Ngian GS, Elford K, Moore O, Stevens W, Nikpour M et al (2016) Mycophenolate mofetil is an effective and safe option for the management of systemic sclerosis-associated interstitial lung disease: results from the Australian Scleroderma Cohort Study. Clin Exp Rheumatol 34(Suppl 1)(5):170–176 PubMed Owen C, Ngian GS, Elford K, Moore O, Stevens W, Nikpour M et al (2016) Mycophenolate mofetil is an effective and safe option for the management of systemic sclerosis-associated interstitial lung disease: results from the Australian Scleroderma Cohort Study. Clin Exp Rheumatol 34(Suppl 1)(5):170–176 PubMed
93.
Zurück zum Zitat Berezne A, Ranque B, Valeyre D, Brauner M, Allanore Y, Launay D et al (2008) Therapeutic strategy combining intravenous cyclophosphamide followed by oral azathioprine to treat worsening interstitial lung disease associated with systemic sclerosis: a retrospective multicenter open-label study. J Rheumatol 35(6):1064–1072 PubMed Berezne A, Ranque B, Valeyre D, Brauner M, Allanore Y, Launay D et al (2008) Therapeutic strategy combining intravenous cyclophosphamide followed by oral azathioprine to treat worsening interstitial lung disease associated with systemic sclerosis: a retrospective multicenter open-label study. J Rheumatol 35(6):1064–1072 PubMed
94.
Zurück zum Zitat Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS et al (2006) A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum 54(12):3962–3970 PubMed Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS et al (2006) A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum 54(12):3962–3970 PubMed
95.
Zurück zum Zitat Poormoghim H, Rezaei N, Sheidaie Z, Almasi AR, Moradi-Lakeh M, Almasi S et al (2014) Systemic sclerosis: comparison of efficacy of oral cyclophosphamide and azathioprine on skin score and pulmonary involvement—a retrospective study. Rheumatol Int 34(12):1691–1699 PubMed Poormoghim H, Rezaei N, Sheidaie Z, Almasi AR, Moradi-Lakeh M, Almasi S et al (2014) Systemic sclerosis: comparison of efficacy of oral cyclophosphamide and azathioprine on skin score and pulmonary involvement—a retrospective study. Rheumatol Int 34(12):1691–1699 PubMed
96.
Zurück zum Zitat Schram ME, Borgonjen RJ, Bik CM, van der Schroeff JG, van Everdingen JJ, Spuls PI et al (2011) Off-label use of azathioprine in dermatology: a systematic review. Arch Dermatol 147(4):474–488 PubMed Schram ME, Borgonjen RJ, Bik CM, van der Schroeff JG, van Everdingen JJ, Spuls PI et al (2011) Off-label use of azathioprine in dermatology: a systematic review. Arch Dermatol 147(4):474–488 PubMed
97.
Zurück zum Zitat Bosello S, De Santis M, Lama G, Spano C, Angelucci C, Tolusso B et al (2010) B cell depletion in diffuse progressive systemic sclerosis: safety, skin score modification and IL-6 modulation in an up to thirty-six months follow-up open-label trial. Arthritis Res Ther 12(2):R54 PubMedPubMedCentral Bosello S, De Santis M, Lama G, Spano C, Angelucci C, Tolusso B et al (2010) B cell depletion in diffuse progressive systemic sclerosis: safety, skin score modification and IL-6 modulation in an up to thirty-six months follow-up open-label trial. Arthritis Res Ther 12(2):R54 PubMedPubMedCentral
98.
Zurück zum Zitat Daoussis D, Liossis SN, Tsamandas AC, Kalogeropoulou C, Kazantzi A, Sirinian C et al (2010) Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology 49(2):271–280 PubMed Daoussis D, Liossis SN, Tsamandas AC, Kalogeropoulou C, Kazantzi A, Sirinian C et al (2010) Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology 49(2):271–280 PubMed
99.
Zurück zum Zitat Jordan S, Distler JH, Maurer B, Huscher D, van Laar JM, Allanore Y et al (2015) Effects and safety of rituximab in systemic sclerosis: an analysis from the European Scleroderma Trial and Research (EUSTAR) group. Ann Rheum Dis 74(6):1188–1194 PubMed Jordan S, Distler JH, Maurer B, Huscher D, van Laar JM, Allanore Y et al (2015) Effects and safety of rituximab in systemic sclerosis: an analysis from the European Scleroderma Trial and Research (EUSTAR) group. Ann Rheum Dis 74(6):1188–1194 PubMed
100.
Zurück zum Zitat Moazedi-Fuerst FC, Kielhauser SM, Brickmann K, Hermann J, Lutfi A, Meilinger M et al (2014) Rituximab for systemic sclerosis: arrest of pulmonary disease progression in five cases. Results of a lower dosage and shorter interval regimen. Scand J Rheumatol 43(3):257–258 PubMedPubMedCentral Moazedi-Fuerst FC, Kielhauser SM, Brickmann K, Hermann J, Lutfi A, Meilinger M et al (2014) Rituximab for systemic sclerosis: arrest of pulmonary disease progression in five cases. Results of a lower dosage and shorter interval regimen. Scand J Rheumatol 43(3):257–258 PubMedPubMedCentral
101.
Zurück zum Zitat Smith V, Van Praet JT, Vandooren B, Van der Cruyssen B, Naeyaert JM, Decuman S et al (2010) Rituximab in diffuse cutaneous systemic sclerosis: an open-label clinical and histopathological study. Ann Rheum Dis 69(1):193–197 PubMed Smith V, Van Praet JT, Vandooren B, Van der Cruyssen B, Naeyaert JM, Decuman S et al (2010) Rituximab in diffuse cutaneous systemic sclerosis: an open-label clinical and histopathological study. Ann Rheum Dis 69(1):193–197 PubMed
102.
Zurück zum Zitat Lafyatis R, Kissin E, York M, Farina G, Viger K, Fritzler MJ et al (2009) B cell depletion with rituximab in patients with diffuse cutaneous systemic sclerosis. Arthritis Rheum 60(2):578–583 PubMedPubMedCentral Lafyatis R, Kissin E, York M, Farina G, Viger K, Fritzler MJ et al (2009) B cell depletion with rituximab in patients with diffuse cutaneous systemic sclerosis. Arthritis Rheum 60(2):578–583 PubMedPubMedCentral
103.
Zurück zum Zitat Keir GJ, Maher TM, Ming D, Abdullah R, de Lauretis A, Wickremasinghe M et al (2014) Rituximab in severe, treatment-refractory interstitial lung disease. Respirology 19(3):353–359 PubMed Keir GJ, Maher TM, Ming D, Abdullah R, de Lauretis A, Wickremasinghe M et al (2014) Rituximab in severe, treatment-refractory interstitial lung disease. Respirology 19(3):353–359 PubMed
104.
Zurück zum Zitat Thiebaut M, Launay D, Riviere S, Mahevas T, Bellakhal S, Hachulla E et al (2018) Efficacy and safety of rituximab in systemic sclerosis: French retrospective study and literature review. Autoimmun Rev 17(6):582–587 PubMed Thiebaut M, Launay D, Riviere S, Mahevas T, Bellakhal S, Hachulla E et al (2018) Efficacy and safety of rituximab in systemic sclerosis: French retrospective study and literature review. Autoimmun Rev 17(6):582–587 PubMed
105.
Zurück zum Zitat Khanna D, Denton CP, Jahreis A, van Laar JM, Frech TM, Anderson ME et al (2016) Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial. Lancet 387(10038):2630–2640 PubMed Khanna D, Denton CP, Jahreis A, van Laar JM, Frech TM, Anderson ME et al (2016) Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial. Lancet 387(10038):2630–2640 PubMed
106.
Zurück zum Zitat Khanna D, Lin CJF, Kuwana M et al (2018) Efficacy and safety of tocilizumab for the treatment of systemic sclerosis: results from a phase 3 randomized controlled trial. 2018 ACR/ARHP annual meeting abstract supplement. Arthritis Rheumatol 70(Suppl 9):898 Khanna D, Lin CJF, Kuwana M et al (2018) Efficacy and safety of tocilizumab for the treatment of systemic sclerosis: results from a phase 3 randomized controlled trial. 2018 ACR/ARHP annual meeting abstract supplement. Arthritis Rheumatol 70(Suppl 9):898
107.
Zurück zum Zitat Khanna D, Spino C, Bush E et al (2018) Abatacept vs. placebo in early diffuse cutaneous systemic sclerosis—results of a phase 2 investigator initiated, double-blind, placebo-controlled, multicenter, randomized controlled trial study. 2018 ACR/ARHP Annual Meeting Abstract Supplement. Arthritis Rheumatol 70(Suppl 9):900 Khanna D, Spino C, Bush E et al (2018) Abatacept vs. placebo in early diffuse cutaneous systemic sclerosis—results of a phase 2 investigator initiated, double-blind, placebo-controlled, multicenter, randomized controlled trial study. 2018 ACR/ARHP Annual Meeting Abstract Supplement. Arthritis Rheumatol 70(Suppl 9):900
108.
Zurück zum Zitat Distler O, Brown KK, Distler JHW, Assassi S, Maher TM, Cottin V et al (2017) Design of a randomised, placebo-controlled clinical trial of nintedanib in patients with systemic sclerosis-associated interstitial lung disease (SENSCIS). Clin Exp Rheumatol 35(Suppl 106(4)):75–81 PubMed Distler O, Brown KK, Distler JHW, Assassi S, Maher TM, Cottin V et al (2017) Design of a randomised, placebo-controlled clinical trial of nintedanib in patients with systemic sclerosis-associated interstitial lung disease (SENSCIS). Clin Exp Rheumatol 35(Suppl 106(4)):75–81 PubMed
109.
Zurück zum Zitat Spiera RF, Hummers LK, Chung L, Frech TM, Domsic RT, Hsu V, Furst DE, Gordon JK, Mayes MD, Simms RW, Constantine S, White B (2017) A phase 2 study of safety and efficacy of anabasum (JBT-101), a cannabinoid receptor type 2 agonist, in diffuse cutaneous systemic sclerosis [abstract. Arthritis Rheumatol 69(suppl 10):2884 Spiera RF, Hummers LK, Chung L, Frech TM, Domsic RT, Hsu V, Furst DE, Gordon JK, Mayes MD, Simms RW, Constantine S, White B (2017) A phase 2 study of safety and efficacy of anabasum (JBT-101), a cannabinoid receptor type 2 agonist, in diffuse cutaneous systemic sclerosis [abstract. Arthritis Rheumatol 69(suppl 10):2884
110.
Zurück zum Zitat Distler O, Allanore Y, Denton CP, Kuwana M, Matucci-Cerinic M, Pope JE, Pena J, Laapas K, Yao Z, Khanna D (2018) Riociguat in patients with early diffuse cutaneous systemic sclerosis: a randomized, double-blind, placebo-controlled phase IIb study (RISE-Ssc) [abstract. Arthritis Rheumatol 70(Suppl 9):Abstract 903 Distler O, Allanore Y, Denton CP, Kuwana M, Matucci-Cerinic M, Pope JE, Pena J, Laapas K, Yao Z, Khanna D (2018) Riociguat in patients with early diffuse cutaneous systemic sclerosis: a randomized, double-blind, placebo-controlled phase IIb study (RISE-Ssc) [abstract. Arthritis Rheumatol 70(Suppl 9):Abstract 903
111.
Zurück zum Zitat Burt RK, Shah SJ, Dill K, Grant T, Gheorghiade M, Schroeder J et al (2011) Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomised phase 2 trial. Lancet 378(9790):498–506 PubMed Burt RK, Shah SJ, Dill K, Grant T, Gheorghiade M, Schroeder J et al (2011) Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomised phase 2 trial. Lancet 378(9790):498–506 PubMed
113.
Zurück zum Zitat van Laar JM, Farge D, Sont JK, Naraghi K, Marjanovic Z, Larghero J et al (2014) Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA 311(24):2490–2498 PubMed van Laar JM, Farge D, Sont JK, Naraghi K, Marjanovic Z, Larghero J et al (2014) Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA 311(24):2490–2498 PubMed
114.
Zurück zum Zitat Henes JC, Schmalzing M, Vogel W, Riemekasten G, Fend F, Kanz L et al (2012) Optimization of autologous stem cell transplantation for systemic sclerosis—a single-center longterm experience in 26 patients with severe organ manifestations. J Rheumatol 39(2):269–275 PubMed Henes JC, Schmalzing M, Vogel W, Riemekasten G, Fend F, Kanz L et al (2012) Optimization of autologous stem cell transplantation for systemic sclerosis—a single-center longterm experience in 26 patients with severe organ manifestations. J Rheumatol 39(2):269–275 PubMed
115.
Zurück zum Zitat Henes JC, Koetter I, Horger M, Schmalzing M, Mueller K, Eick C et al (2014) Autologous stem cell transplantation with thiotepa-based conditioning in patients with systemic sclerosis and cardiac manifestations. Rheumatology 53(5):919–922 PubMed Henes JC, Koetter I, Horger M, Schmalzing M, Mueller K, Eick C et al (2014) Autologous stem cell transplantation with thiotepa-based conditioning in patients with systemic sclerosis and cardiac manifestations. Rheumatology 53(5):919–922 PubMed
116.
Zurück zum Zitat Sullivan KM, Goldmuntz EA, Keyes-Elstein L, McSweeney PA, Pinckney A, Welch B et al (2018) Myeloablative autologous stem-cell transplantation for severe Scleroderma. N Engl J Med 378(1):35–47 PubMedPubMedCentral Sullivan KM, Goldmuntz EA, Keyes-Elstein L, McSweeney PA, Pinckney A, Welch B et al (2018) Myeloablative autologous stem-cell transplantation for severe Scleroderma. N Engl J Med 378(1):35–47 PubMedPubMedCentral
117.
Zurück zum Zitat Burt RK, Oliveira MC, Shah SJ, Moraes DA, Simoes B, Gheorghiade M et al (2013) Cardiac involvement and treatment-related mortality after non-myeloablative haemopoietic stem-cell transplantation with unselected autologous peripheral blood for patients with systemic sclerosis: a retrospective analysis. Lancet 381(9872):1116–1124 PubMed Burt RK, Oliveira MC, Shah SJ, Moraes DA, Simoes B, Gheorghiade M et al (2013) Cardiac involvement and treatment-related mortality after non-myeloablative haemopoietic stem-cell transplantation with unselected autologous peripheral blood for patients with systemic sclerosis: a retrospective analysis. Lancet 381(9872):1116–1124 PubMed
118.
Zurück zum Zitat Walker UA, Saketkoo LA, Distler O (2018) Haematopoietic stem cell transplantation in systemic sclerosis. RMD Open 4(1):e533 PubMedPubMedCentral Walker UA, Saketkoo LA, Distler O (2018) Haematopoietic stem cell transplantation in systemic sclerosis. RMD Open 4(1):e533 PubMedPubMedCentral
119.
Zurück zum Zitat Farge D, Burt RK, Oliveira MC, Mousseaux E, Rovira M, Marjanovic Z et al (2017) Cardiopulmonary assessment of patients with systemic sclerosis for hematopoietic stem cell transplantation: recommendations from the European Society for Blood and Marrow Transplantation Autoimmune Diseases Working Party and collaborating partners. Bone Marrow Transplant 52(11):1495–1503 PubMedPubMedCentral Farge D, Burt RK, Oliveira MC, Mousseaux E, Rovira M, Marjanovic Z et al (2017) Cardiopulmonary assessment of patients with systemic sclerosis for hematopoietic stem cell transplantation: recommendations from the European Society for Blood and Marrow Transplantation Autoimmune Diseases Working Party and collaborating partners. Bone Marrow Transplant 52(11):1495–1503 PubMedPubMedCentral
Metadaten
Titel
Systemische Sklerose – klinisches Bild, Diagnostik und Therapie
verfasst von
Dr. med. M. O. Becker
O. Distler
B. Maurer
Publikationsdatum
14.05.2019

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