[2009] [FRI0324] ASSOCIATED AUTOIMMUNE DISEASES IN SYSTEMIC SCLEROSIS DEFINE A SUBSET OF PATIENTS WITH MILDER DISEASE: RESULTS FROM TWO LARGE COHORTS OF EUROPEAN CAUCASIAN PATIENTS
J. Avouac1, P. Airò2, P. Dieude3, P. Caramaschi4, K. Tiev5, E. Diot6, J. Sibilia7, S. Cappelli8, B. Granel9, A. Vacca10, J. Wipff1, O. Meyer3, A. Kahan1, M. Matucci-Cerinic8, Y. Allanore1 1Rheumatology A, Cochin Hospital, Paris, France; 2Rheumatology and Clinical Immunology, Brescia, Italy; 3Rheumatology department, Bichat Claude Bernard Hospital, Paris, France; 4Rheumatology department, Verona, Italy; 5Internal Medicine department, Saint Antoine Hospital, Paris; 6INSERM EMI-U 00-10, Tours; 7Rheumatology department, Hautepierre Hospital, Strasbourg, France; 8Rheumatology department, Florence, Italy; 9Internal Medicine department, Marseille, France; 10IInd Chair of Rheumatology, Cagliary, Italy
Background: Auto-immune background of systemic sclerosis (SSc) remains disputed. Recent data suggested that associated auto-immune diseases (AID) could be common in SSc but their impact on disease phenotype remain unclear.
Objectives: To assess the prevalence and potential influence of AID on SSc phenotype.
Methods: A multicenter study was performed in France and Italy to recruit consecutive European Caucasian SSc patients systematically assessed for the co-existence of predefined AID known to occur with connective tissue diseases. These latter were defined in accordance with standard international criteria and the following were screened: Sjogren syndrome (SS), thyroiditis, primary biliary cirrhosis (PBC), myositis, rheumatoid arthritis (RA), systemic lupus erythematosus (SLE).
We recruited 585 (85% females) and 547 (90% females) SSc patients from France and Italy respectively. The mean age was 57±14 and 63±14 years old in the French and Italian cohort respectively (p<0.0001) and the mean disease duration was 11±9 and 13±8 years respectively (p<0.0001). The frequency of limited cutaneous subtype (75% vs 67%, p=0.004), antinuclear antibodies (99% vs. 86%, p<0.0001), anticentromere antibodies (50% vs. 33%, p<0.0001), altered carbon monoxide lung diffusion (DLCO/VA<75%; 52% vs. 32%, p<0.0001) was significantly higher in the Italian cohort.
Results: AID were found in 114/585 (19%) and 179/547 (33%) SSc patients (p<0.0001) from France and Italy respectively. SS and thyroiditis were the predominant AID in both cohorts but with higher prevalence in the Italian one (17% vs. 7.5%, p<0.0001 for SS and 8.5% vs. 4%, p<0.0001 for thyroiditis). The frequency of PBC, myositis, RA and SLE were similar.
The co-existence of at least one AID in the whole cohort was associated in multivariate analysis with the limited cutaneous subtype (p=0.02), the presence of antinuclear antibodies (p=0.002) and a lower prevalence of digital ulcers (p=0.02). SS (137/1132, 12%) was associated with the limited cutaneous subtype (p=0.002) and the presence of antinuclear antibodies (p=0.01). There was a trend for higher prevalence among females and lower frequency of digital ulcers in patients with autoimmune thyroiditis (70/1132, 6%). PBC (31/1132, 3%) was associated with the presence of anticentromere antibodies (p=0.03) and myositis with the use of immunosuppressive drugs (p<0.0001).
Conclusion: Our study shows that about a quarter of this large series of European Caucasian patients affected by SSc has developed one or more AID known to occur with connective tissue diseases. Moreover, presence of at least one of these AID was associated with the presence of antinuclear antibodies, the limited cutaneous subtype and lower frequency of digital ulceration. This defines a subset of patients with a milder disease, probably associated with a specific B-cell-mediated autoimmunity and weaker fibrotic or vascular propensities. This highly suggests the implication of genetic background that would drive predominant autoimmunity. Therefore association of AID in SSc has to be considered for investigations in SSc and in particular for genetic, immune or therapeutic studies.
Disclosure of Interest: None declared
Ann Rheum Dis 2009;68(Suppl3):463
Scleroderma, myositis and related syndromes
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