{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T06:01:03Z","timestamp":1792735263336,"version":"4.5.0"},"reference-count":37,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2020,1,28]],"date-time":"2020-01-28T00:00:00Z","timestamp":1580169600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2020,3]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To describe the associations between autoantibodies, clinical presentation, and outcomes among patients with systemic sclerosis (<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c) in order to develop a novel <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c classification scheme that would incorporate both antibodies and the cutaneous disease subset as criteria.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Demographic and clinical characteristics, including cutaneous subset, time of disease and organ complication onset, and autoantibody specificities, were determined in a cohort of SSc subjects. Survival analysis was used to assess the effect of the autoantibodies on organ disease and death.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The study included 1,325 subjects. Among the antibody\/skin disease subsets, anticentromere antibody\u2013positive patients with limited cutaneous <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c (lc<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c) (n = 374) had the highest 20\u2010year survival (65.3%), lowest incidence of clinically significant pulmonary fibrosis (<jats:styled-content style=\"fixed-case\">PF<\/jats:styled-content>) (8.5%) and scleroderma renal crisis (<jats:styled-content style=\"fixed-case\">SRC<\/jats:styled-content>) (0.3%), and lowest incidence of cardiac <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c (4.9%), whereas the frequency of pulmonary hypertension (<jats:styled-content style=\"fixed-case\">PH<\/jats:styled-content>) was similar to the mean value in the <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c cohort overall. The anti\u2013Scl\u201070+ groups of patients with lc<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c (n = 138) and patients with diffuse cutaneous <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c (dc<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c) (n = 149) had the highest incidence of clinically significant <jats:styled-content style=\"fixed-case\">PF<\/jats:styled-content> (86.1% and 84%, respectively, at 15 years). Anti\u2010Scl\u201070+ patients with dc<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c had the lowest survival (32.4%) and the second highest incidence of cardiac <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c (12.9%) at 20 years. In contrast, in anti\u2010Scl\u201070+ patients with lc<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c, other complications were rare, and these patients demonstrated the lowest incidence of <jats:styled-content style=\"fixed-case\">PH<\/jats:styled-content> (6.9%) and second highest survival (61.8%) at 20 years. Anti\u2013<jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content> polymerase antibody\u2013positive <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients (n = 147) had the highest incidence of <jats:styled-content style=\"fixed-case\">SRC<\/jats:styled-content> (28.1%) at 20 years. The anti\u2013U3 <jats:styled-content style=\"fixed-case\">RNP<\/jats:styled-content>+ <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c group (n = 56) had the highest incidence of <jats:styled-content style=\"fixed-case\">PH<\/jats:styled-content> (33.8%) and cardiac <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c (13.2%) at 20 years. Among lc<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients with other autoantibodies (n = 295), the risk of <jats:styled-content style=\"fixed-case\">SRC<\/jats:styled-content> and cardiac <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c was low at 20 years (2.7% and 2.4%, respectively), while the frequencies of other outcomes were similar to the mean values in the full <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c cohort. Patients with dc<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c who were positive for other autoantibodies (n = 166) had a poor prognosis, demonstrating the second lowest survival (33.6%) and frequent organ complications.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>These findings highlight the importance of autoantibodies, cutaneous subset, and disease duration when assessing morbidity and mortality in patients with <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c. Our novel classification scheme may improve disease monitoring and benefit future clinical trial designs in <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.41153","type":"journal-article","created":{"date-parts":[[2019,11,4]],"date-time":"2019-11-04T06:06:28Z","timestamp":1572847588000},"page":"465-476","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":210,"title":["Using Autoantibodies and Cutaneous Subset to Develop Outcome\u2010Based Disease Classification in Systemic Sclerosis"],"prefix":"10.1002","volume":"72","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5178-9038","authenticated-orcid":false,"given":"Svetlana I.","family":"Nihtyanova","sequence":"first","affiliation":[{"name":"UCL Division of Medicine Royal Free Hospital  London 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