{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T02:43:02Z","timestamp":1790390582065,"version":"4.1.0"},"reference-count":20,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2017,5,30]],"date-time":"2017-05-30T00:00:00Z","timestamp":1496102400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","award":["K23\u2010AR\u2010061439"],"award-info":[{"award-number":["K23\u2010AR\u2010061439"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","award":["P30\u2010AR\u2010053503"],"award-info":[{"award-number":["P30\u2010AR\u2010053503"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100001845","name":"Scleroderma Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001845","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2017,6]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Prior studies have demonstrated an increased risk of cancer\u2010associated scleroderma in patients with anti\u2013RNA polymerase III (anti\u2013RNAP III) autoantibodies as well as in patients who are triple\u2010negative for anticentromere (anti\u2010CENP), anti\u2013topoisomerase I (anti\u2013topo I), and anti\u2013RNAP III (also known as anti\u2010POL) autoantibodies (referred to as CTP negative). In a recent study of 16 CTP\u2010negative scleroderma patients with coincident cancer, 25% of the patients were found to have autoantibodies to RNPC\u20103, a member of the minor spliceosome complex. This investigation was undertaken to validate the relationship between anti\u2013RNPC\u20103 antibodies and cancer and examine the associated clinical phenotype in a large sample of scleroderma patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Scleroderma patients with cancer were assayed for anti\u2010CENP, anti\u2013topo I, anti\u2013RNAP III, and anti\u2013RNPC\u20103 autoantibodies. Disease characteristics and the cancer\u2013scleroderma interval were compared across autoantibody groups. The relationship between autoantibody status and cancer\u2010associated scleroderma was assessed by logistic regression.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 318 patients with scleroderma and cancer, 70 (22.0%) were positive for anti\u2013RNAP III, 54 (17.0%) were positive for anti\u2013topo I, and 96 (30.2%) were positive for anti\u2010CENP. Twelve patients (3.8% of the overall group or 12.2% of CTP\u2010negative patients) were positive for anti\u2013RNPC\u20103. Patients with anti\u2013RNPC\u20103 had a short cancer\u2013scleroderma interval (median 0.9 years). Relative to patients with anti\u2010CENP, patients with anti\u2013RNPC\u20103 and those with anti\u2013RNAP III had a &gt;4\u2010fold increased risk of cancer within 2 years of scleroderma onset (for anti\u2013RNPC\u20103\u2013positive patients, odds ratio [OR] 4.3, 95% confidence interval [95% CI] 1.10\u201316.9 [<jats:italic>P<\/jats:italic>\u2009=\u20090.037]; for anti\u2013RNAP III\u2013positive patients, OR 4.49, 95% CI 1.98\u201310.2 [<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001]). Patients with anti\u2013RNPC\u20103 had severe restrictive lung disease, gastrointestinal disease, Raynaud's phenomenon, and myopathy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Anti\u2013RNPC\u20103 autoantibodies, similar to anti\u2013RNAP III autoantibodies, are associated with an increased risk of cancer at the onset of scleroderma. These data suggest the possibility of cancer\u2010induced autoimmunity in this subset of patients with scleroderma.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.40065","type":"journal-article","created":{"date-parts":[[2017,2,19]],"date-time":"2017-02-19T22:56:42Z","timestamp":1487545002000},"page":"1306-1312","source":"Crossref","is-referenced-by-count":79,"title":["Brief Report: Anti\u2013RNPC\u20103 Antibodies As a Marker of Cancer\u2010Associated Scleroderma"],"prefix":"10.1002","volume":"69","author":[{"given":"Ami A.","family":"Shah","sequence":"first","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"George","family":"Xu","sequence":"additional","affiliation":[{"name":"Harvard University and Harvard\u2013Massachusetts Institute of Technology Division of Health Sciences and Technology, Cambridge, Massachusetts, and Howard Hughes Medical Institute and Brigham and Women's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antony","family":"Rosen","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura K.","family":"Hummers","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fredrick M.","family":"Wigley","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen J.","family":"Elledge","sequence":"additional","affiliation":[{"name":"Howard Hughes Medical Institute, Brigham and Women's Hospital, and Harvard University Medical School Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Livia","family":"Casciola\u2010Rosen","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,5,30]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.37969"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.27549"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.39022"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.101144"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1186\/ar4486"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1186\/ar3544"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1246886"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38928"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/nbt.1856"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/nbt.2539"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1615990113"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.emboj.7600765"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1261\/rna.1359909"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.24586"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38098"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780230510"},{"key":"e_1_2_7_18_1","first-page":"202","article-title":"Scleroderma (systemic sclerosis): classification, subsets and pathogenesis","volume":"15","author":"LeRoy EC","year":"1988","journal-title":"J Rheumatol"},{"key":"e_1_2_7_19_1","first-page":"1892","article-title":"Skin thickness score in systemic sclerosis: an assessment of interobserver variability in 3 independent studies","volume":"20","author":"Clements PJ","year":"1993","journal-title":"J Rheumatol"},{"key":"e_1_2_7_20_1","first-page":"2159","article-title":"A disease severity scale for systemic sclerosis: development and testing","volume":"26","author":"Medsger TA","year":"1999","journal-title":"J Rheumatol"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38093"}],"container-title":["Arthritis &amp; 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