• Florian M P Meier, Klaus W Frommer, Robert Dinser, Ulrich A Walker, Laszlo Czirjak, Christopher P Denton, Yannick Allanore, Oliver Distler, Gabriela Riemekasten, Gabriele Valentini, Ulf Müller-Ladner, and EUSTAR Co-authors.
• Department of Internal Medicine and Rheumatology, Justus-Liebig-University Gießen Kerckhoff-Klinik, Bad Nauheim, Germany.
• Ann. Rheum. Dis. 2012 Aug 1; 71 (8): 1355-60.

ObjectivesSystemic sclerosis (SSc) is a rare disease requiring multicentre collaboration to reveal comprehensive details of disease-related causes for morbidity and mortality.MethodsThe European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) group initiated a database to prospectively gather key data of patients with SSc using a minimal essential dataset that was reorganised in 2008 introducing new items. Baseline visit data of patients who were registered between 2004 and 2011 were analysed using descriptive statistics.ResultsIn June 2011, 7655 patients (2838 with diffuse cutaneous (dc) and 4481 with limited cutaneous (lc) SSc who fulfilled the American College of Rheumatology diagnostic criteria had been registered in 174 centres, mainly European. The most prominent hallmarks of disease were Raynaud's phenomenon (96.3%), antinuclear antibodies (93.4%) and a typical capillaroscopic pattern (90.9%). Scleroderma was more common on fingers and hands than on any other part of the skin. Proton pump inhibitors (65.2%), calcium channel blockers (52.7%), and corticosteroids (45.3%) were most often prescribed. Among the immunosuppressant agents, cyclophosphamide was used more often in dcSSc than in lcSSc.ConclusionsThe EUSTAR database provides an abundance of information on the true clinical face of SSc that will be helpful in improving the classification of SSc and its subsets and for developing more specific therapeutic recommendations.

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