ANALYSIS OF POLIAUTOIMMUNITY IN THE DIFFERENT SUBSETS OF SCLERODERMA

Background: Coexistence of different connective tissue diseases based on their common autoimmune base is a feasible circumstance, which is known as poliautoimmunity (PAI). Objectives: Evaluation of the occurrence of PAI in systemic sclerosis (SSc) according to subtypes in the patients included in the Spanish SSc Registry (RESCLE). Causes of death were also analyzed. Methods: A nationwide, cross-sectional study was carried out. All participating centers had obtained local ethics committee approval. Results: PAI was present in 46% out of 1911 patients, of whom, 33% had more than one association. Most of these patients were women (93%, p<0.001), with no significant differences regarding first manifestation: Raynaud’s phenomenon, puffy hands, arthralgia or skin sclerosis. Isolated SSc was more frequent in all subsets ( table 1 ). Sjogren’s syndrome was by far the most frequent associated disorder, with a RR of 1376 ( table 2 ). Causes of death (SSc-related or not) did not significantly differ whether PAI was present or not. Table 1 Prevalence of PAI according to subsets. Isolated SSc PAI P-VALUE lcSSc 588 (57%) 554 (63%) 0.008 dcSSc 210 (20%) 188 (21%) 0.611 ssSSc 121 (12%) 85 (9.7%) 0.160 earlySSc 28 (2.7%) 16 (1.8%) 0.222 preSSc 85 (8.2%) 36 (4.1%) <0.001 Table 2 Prevalence of the different associated autoimmune disorders. TOTAL lcSSc dcSSc sineSSC earlySSC preSSc Sjögren’s síndrome 475 (25%) 317 (28%) 106 (27%) 34 (17%) 6 (14%) 12 (9.9%) Thyroid autoimmune disorder 268 (14%) 173 (15%) 36 (9.0%) 35 (17%) 8 (18%) 16 (13%) Primary biliary colangitis 88 (4.6%) 61 (5.3%) 5 (1.3%) 17 (8.3%) 0 (0.00%) 5 (4.1%) Sclerosing colangitis 6 (0.31%) 4 (0.35%) 0 (0.00%) 0 (0.00%) 0 (0.00%) 2 (1.7%) Autoimmune hepatitis 20 (1.0%) 17 (1.5%) 2 (0.50%) 0 (0.00%) 0 (0.00%) 1 (0.83%) Systemic lupus erythematosus 11 (0.58%) 7 (0.61%) 1 (0.25%) 3 (1.5%) 0 (0.00%) 0 (0.00%) Antiphospholipid syndrome 16 (0.84%) 8 (0.70%) 5 (1.3%) 2 (0.97%) 1 (2.3%) 0 (0.00%) Inflammatory myopathy 99 (5.2%) 45 (3.9%) 47 (12%) 6 (2.9%) 1 (2.3%) 0 (0.00%) Rheumatoid arthritis 6 (0.31%) 4 (0.35%) 2 (0.50%) 0 (0.00%) 0 (0.00%) 0 (0.00%) Conclusion: A rather higher prevalence than reported was observed, although distribution of the associated disorders was similar. No remarkable differences were found regarding SSc subsets, either PAI prevalence, initial manifestations or causes of death. Disclosure of Interests: None declared DOI: 10.1136/annrheumdis-2019-eular.4870Citation: Ann Rheum Dis, volume 78, supplement 2, year 2019, page A1224Session: Scleroderma, myositis and related syndromes (Scientific Abstracts)