Background: Systemic sclerosis (SSc) is a vasculopathy with increased tissue deposition of collagen. The aetiology is unknown. Genetic and environmental susceptibility factors have been implicated. It is unknown whether or not disease presentation varies within Europe.
Aims and Methods: Baseline data of all SSc patients entered in the EULAR Scleroderma Trials And Research (EUSTAR) database up to April 2007 were analyzed for geographic differences with regard to organ involvement, and geographic clusters with regard to clinical subsets (diffuse vs. limited SSc) and autoantibodies (anti-centromere vs. anti-Scl70).
Results: 3,661 patients from 79 centres in 62 cities and 23 countries were analyzed. There was no clear trend between geographical coordinates and SSc subsets, although there appeared to be an increased prevalence of Scl70 in the more eastern centres. There was no association between geographical longitude or latitude and the age at the onset of Raynaud’s phenomenon or the onset of non-Raynaud’s symptoms. There was also a trend for the more eastern centres to care for patients with a higher prevalence of more severe organ manifestations (pulmonary arterial hypertension, cardiac involvement). Between different centres within one city, there was a large variability in the frequency of organ complications.
Conclusion: Our analysis suggests that eastern centres care for more severe SSc manifestations in Europe. Large differences in patient referral account for a large local variability of SSc presentations and preclude the identification of genetic or environmental factors.