DMW – Klinischer Fortschritt, Table of Contents Dtsch Med Wochenschr 2015; 140(07): 504-507DOI: 10.1055/s-0041-100929 Klinischer Fortschritt Rheumatologie © Georg Thieme Verlag KG Stuttgart · New York Systemische Sklerose – ein Update Systemic sclerosis – an update Christina Bergmann Medizinische Klinik 3, Universität Erlangen-Nürnberg , Jörg H. W. Distler Medizinische Klinik 3, Universität Erlangen-Nürnberg › Author Affiliations Recommend Article Abstract Buy Article Schlüsselwörter Schlüsselwörtersystemische Sklerose - Klassifikation - antifibrotische Therapie Keywords Keywordssystemic sclerosis - classification - antifibrotic treatment Full Text References Literatur 1 Pulido T, Adzerikho I, Channick RN et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369: 809-818 2 Nikpour M, Baron M. Mortality in systemic sclerosis: lessons learned from population-based and observational cohort studies. Curr Opin Rheumatol 2014; 26: 131-137 3 Gabrielli A, Avvedimento EV, Krieg T. Scleroderma. N Engl J Med 2009; 360: 1989-2003 4 Varga J, Abraham D. Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest 2007; 117: 557-567 5 Beyer C, Zenzmaeier C, Palumbo-Zerr K et al. Stimulation of the soluble guanylate cyclase (sGC) inhibits fibrosis by blocking non-canonical TGFbeta signalling. Ann Rheum Dis 2014; 6 van den Hoogen F, Khanna D, Fransen J et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology / European league against rheumatism collaborative initiative. Ann Rheum Dis 2013; 72: 1747-1755 7 Andreasson K, Saxne T, Bergknut C et al. 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 2014; 73: 1788-1792 8 Matucci-Cerinic M, Allanore Y, Czirják L et al. The challenge of early systemic sclerosis for the EULAR Scleroderma Trial and Research group (EUSTAR) community. It is time to cut the Gordian knot and develop a prevention or rescue strategy. Ann Rheum Dis 2009; 68: 1377-1380 9 Minier T, Guiducci S, Bellando-Randone S et al. 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 2013; 73: 2087-2093 10 Jordan S, Distler JH, Maurer B et al. Effects and safety of rituximab in systemic sclerosis: an analysis from the European Scleroderma Trial and Research (EUSTAR) group. Ann Rheum Dis 2014; 11 Richeldi L, du Bois RM, Raghu G et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2071-2082 12 Noble PW, Albera C, Bradford WZ et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 2011; 377: 1760-1769 13 Humbert M, Yaici A, de Groote P et al. Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum 2011; 63: 3522-3530 14 Coghlan JG, Denton CP, Grünig E et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 2014; 73: 1340-349 15 Ghofrani HA, Galiè N, Grimminger F et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013; 369: 330-340