{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,12]],"date-time":"2026-08-12T17:15:43Z","timestamp":1786554943285,"version":"build-2736575974"},"reference-count":37,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2022,7,21]],"date-time":"2022-07-21T00:00:00Z","timestamp":1658361600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["P50\u2010AR\u2010060780"],"award-info":[{"award-number":["P50\u2010AR\u2010060780"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>This study was undertaken to describe clinical manifestations in patients with Th\/To antibody\u2013positive systemic sclerosis (SSc) during long\u2010term follow\u2010up.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We performed a case\u2013control study involving anti\u2010Th\/To antibody\u2013positive patients with SSc who were newly referred to the University of Pittsburgh Medical Center and the Pittsburgh Scleroderma Center from 1980 to 2015. For every case, 2 anti\u2010Th\/To antibody\u2013negative SSc patients (the first 2 consecutively seen after a case) were used as controls. Long\u2010term disease manifestations and survival were then compared between cases and controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 204 anti\u2010Th\/To antibody\u2013positive SSc patients and 408 controls were identified. The cohort had a mean\u2009\u00b1\u2009SD age of 52\u2009\u00b1\u200912.9\u2009years, and 76% of individuals were women. Anti\u2010Th\/To antibody\u2013positive patients more often presented without skin thickening (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.0001) and had a higher rate of pulmonary hypertension (PH) (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.0001) and interstitial lung disease (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.05) compared to anti\u2010Th\/To antibody\u2013negative SSc controls. Anti\u2010Th\/To antibody\u2013positive SSc patients also had less frequent muscle and joint involvement than anti\u2010Th\/To antibody\u2013negative SSc controls (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.0001). After a median clinical follow\u2010up period of 6.1\u2009years (interquartile range 2.4\u201312.7), 38% of anti\u2010Th\/To\u2013positive patients had developed PH compared to 15% of anti\u2010Th\/To antibody\u2013negative SSc controls (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.0001). The rate of PH classified as World Health Organization (WHO) Group 1 pulmonary arterial hypertension [PAH] was 23% in anti\u2010Th\/To\u2013positive patients compared to 9% in anti\u2010Th\/To antibody\u2013negative SSc controls (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.0001). After adjusting for age and sex, anti\u2010Th\/To antibody positivity was associated with a hazard ratio (HR) of 3.3 (95% confidence interval 2.3\u20134.9) for increased risk of developing PH at 10\u2009years of follow\u2010up from the first scleroderma center visit.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This is the largest cohort of patients with anti\u2010Th\/To antibody\u2013positive SSc with long\u2010term follow\u2010up data. The very high rate (38%) and associated independent risk of anti\u2010Th\/To antibody\u2013positive patients developing PH in follow\u2010up, particularly in WHO Group 1 PAH patients, is striking. Patients presenting with limited skin involvement should be tested for Th\/To antibodies, and if present, careful monitoring for PH is warranted.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.42152","type":"journal-article","created":{"date-parts":[[2022,4,25]],"date-time":"2022-04-25T10:26:16Z","timestamp":1650882376000},"page":"1580-1587","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Development of Pulmonary Hypertension in Over One\u2010Third of Patients With Th\/To Antibody\u2013Positive Scleroderma in Long\u2010Term Follow\u2010Up"],"prefix":"10.1002","volume":"74","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5429-8899","authenticated-orcid":false,"given":"Shashank","family":"Suresh","sequence":"first","affiliation":[{"name":"University of Pittsburgh Medical Center  Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Devon","family":"Charlton","sequence":"additional","affiliation":[{"name":"University of Pittsburgh School of Medicine  Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erin K.","family":"Snell","sequence":"additional","affiliation":[{"name":"Northwest Permanente  Portland Oregon"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maureen","family":"Laffoon","sequence":"additional","affiliation":[{"name":"University of Pittsburgh School of Medicine  Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr","given":"Thomas A.","family":"Medsger","sequence":"additional","affiliation":[{"name":"University of Pittsburgh School of Medicine  Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lei","family":"Zhu","sequence":"additional","affiliation":[{"name":"University of Pittsburgh School of Medicine  Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2765-0922","authenticated-orcid":false,"given":"Robyn T.","family":"Domsic","sequence":"additional","affiliation":[{"name":"University of Pittsburgh School of Medicine  Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,7,21]]},"reference":[{"key":"e_1_2_7_2_1","first-page":"1633","volume-title":"Arthritis and allied conditions: a textbook of rheumatology","author":"Silver 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