RESEARCH - Clinical risk assessment of organ manifestations in systemic sclerosis - EUSTAR group database

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Ann Rheum Dis. Published Online First: 18 January 2007.

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Extended Report

Clinical risk assessment of organ manifestations in systemic sclerosis - a

report from the EULAR Scleroderma Trials And Research (EUSTAR) group data

base

U A 1*, A Tyndall 1, L Czirják 2, C P Denton 3, D Farge Bancel 4, O

Kowal-Bielecka 5, U Müller-Ladner 6, C Bocelli-Tyndall 1 and M

Matucci-Cerinic 7

1 Basle University Dept. of Rheumatology, Switzerland

2 University of Pécs, Hungary

3 Royal Free and University College London Medical School, United Kingdom

4 Hopital Saint Louis, Paris, France

5 Medical University of Bialystok, Poland

6 Kerckhoff-Klinik Bad Nauheim, Germany

7 University of Florence, Italy

Abstract

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 ACR diagnostic criteria

in participating centres. We aimed to characterize 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 female. On multivariate analysis, scleroderma subsets

(dcSSc vs. lcSSc), antibody status and age at onset of Raynaud's phenomenon,

but not gender were independently associated with the prevalence of organ

manifestations. Autoantibody status in this analysis appeared 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 appeared

superseded by an antibody based classification in predicting some

scleroderma complications. The EUSTAR MEDS data base facilitates the

analysis of clinical patterns in SSc and contributes to the standardised

assessment and monitoring of SSc internationally.

http://ard.bmj.com/cgi/content/abstract/ard.2006.062901v2

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