PT  - JOURNAL ARTICLE
AU  - U A Walker
AU  - A Tyndall
AU  - L Czirják
AU  - C Denton
AU  - D Farge-Bancel
AU  - O Kowal-Bielecka
AU  - U Müller-Ladner
AU  - C Bocelli-Tyndall
AU  - M Matucci-Cerinic
ED  - ,
TI  - Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database
AID  - 10.1136/ard.2006.062901
DP  - 2007 Jun 01
TA  - Annals of the Rheumatic Diseases
PG  - 754--763
VI  - 66
IP  - 6
4099  - http://ard.bmj.com/content/66/6/754.short
4100  - http://ard.bmj.com/content/66/6/754.full
SO  - Ann Rheum Dis2007 Jun 01; 66
AB  - Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (lcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004.Aims and methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits.Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with lcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs lcSSc), antibody status and age at onset of Raynaud’s phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets.Conclusion: dcSSc and lcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally.