[2009] [THU0298] PREVALENCE OF ASSOCIATED AUTO-IMMUNE DISEASES IN PATIENTS WITH SYSTEMIC SCLEROSIS. AN ITALIAN MONOCENTRIC CASISTIC

R. La Corte, A. Lo Monaco, S. Volpinari, M. Bruschi, F. Trotta Rheumatology Unit, S. Anna Hospital, Ferrara, Italy

Background: Systemic sclerosis (SSc) is a connective tissue disease that may present concomitantly with other systemic connective or organ specific autoimmune tissue diseases. These associations are well described for systemic lupus erythematosus and rheumatoid arthritis. The presence of SSc may influence the phenotypic expression of other autoimmune disease and vice versa and different associations could be preferentially related to different subsets of SSc.
Objectives: This study was performed to determine the presence of associated autoimmune diseases in a large population of SSc patients consecutively collected from our database in the last 12 years.
Autoimmune associated diseases (AAD) were defined in accordance with standard international criteria. The frequency of AAD and the phenotype influence were determined.
Methods: We collected 499 SSc patients (94.2% women). The mean age at the diagnosis was 57.1±6.2 years and the mean disease duration was 126±11.9 months. The Limited subset was present in 430 patients (86.1%) and the Diffuse subset in 69 patients (13.8%). ANA were found in all patients; specific SSc autoantibodies anti centromere (ACA) and anti topoisomerasi 1 were found in 262 (52.5%) and in 102 (20.4%) patients respectively. Other non organ specific autoantibodies were found in 53 (10.6%) patients.
Results: 156 patients (31.2%) had at least one AAD: Sjogren's syndrome (18.4%), thyroiditis (7.2%), biliary chirrosis (5.4%), SLE (2.2%) and RA (2.0%) were the more frequent associated diseases. Thirty-five patients had more than 1 associated disease; the more frequent associations were Sjogren's Syndrome with biliary chirrosis (7 cases) and Sjogren' s syndrome with thyroiditis (8 cases). In the 69 patients with Diffuse disease, 15 (21.7%) had one AAD, while in the 430 patients with Limited subset, 147 presented one AAD (34.1%) with a difference statistically significant (p<0.05). Biliary chirrosis was found exclusively in limited subset and in 11/27 cases was also associated with Sjogren's syndrome (Reynolds's syndrome). Other associations were not differently distributed between the two subset of SSc. The presence of ACA was statistically associated with the presence of AAD (p=0.02) while anti-topoisomerasi 1 antibodies were inversely correlated with AAD (P<0.0001).
No differences was found between the presence of an AAD and the occurrence of digital ulcer, lung involvement, esophageal dysfunction and calcinosis.
Conclusion: Sjogren's syndrome and biliary chirrosis appear the more frequent associated diseases. The frequency of thyroiditis is not superior to the prevalence in the general population. The limited subset of SSc is more frequently associated with AAD.
Disclosure of Interest: None declared

Ann Rheum Dis 2009;68(Suppl3):270

Scleroderma, myositis and related syndromes