{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T05:51:10Z","timestamp":1791438670196,"version":"4.3.3"},"reference-count":46,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2023,2,17]],"date-time":"2023-02-17T00:00:00Z","timestamp":1676592000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100001845","name":"Scleroderma Research Foundation","doi-asserted-by":"publisher","award":["K23-AR-071473"],"award-info":[{"award-number":["K23-AR-071473"]}],"id":[{"id":"10.13039\/100001845","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["P30 AR070254"],"award-info":[{"award-number":["P30 AR070254"]}],"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 Care &amp; Research"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>The gastrointestinal tract is commonly involved in patients with systemic sclerosis (SSc) with varied manifestations. As our understanding of SSc gastrointestinal disease pathogenesis and risk stratification is limited, we sought to investigate whether patterns of esophageal dysfunction associate with specific clinical phenotypes in SSc.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients enrolled in the Johns Hopkins Scleroderma Center Research Registry who completed high\u2010resolution esophageal manometry (HREM) studies as part of their clinical care between 2011 and 2020 were identified. Associations between esophageal abnormalities on HREM (absent contractility [AC], ineffective esophageal motility [IEM], hypotensive lower esophageal sphincter [hypoLES]) and patient demographic information, clinical characteristics, and autoantibody profiles were examined.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Ninety\u2010five patients with SSc had HREM data. Sixty\u2010five patients (68.4%) had AC (37 patients with only AC, 28 patients with AC and a hypoLES), 9 patients (9.5%) had IEM, and 11 patients (11.6%) had normal studies. AC was significantly associated with diffuse cutaneous disease (38.5% versus 10.0%; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.01), more severe Raynaud's phenomenon, including digital pits, ulcers, or gangrene (56.9% versus 30.0%; <jats:italic>P<\/jats:italic>\u00a0=\u00a00.02), and reduced median diffusing capacity of lung for carbon monoxide (50.6% versus 72.2%; <jats:italic>P<\/jats:italic>\u00a0=\u00a00.03). AC was observed in most of the patients who died (13 of 14; <jats:italic>P<\/jats:italic>\u00a0=\u00a00.06). These findings were not seen in patients with IEM.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Among patients with SSc, AC is associated with a significantly more severe clinical phenotype. IEM may associate with a milder phenotype. Further studies are needed to evaluate AC, IEM, and their clinical impact relative to the timing of other end\u2010organ complications in SSc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.25080","type":"journal-article","created":{"date-parts":[[2022,12,28]],"date-time":"2022-12-28T05:25:19Z","timestamp":1672205119000},"page":"1715-1724","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Associations Between Patterns of Esophageal Dysmotility and Extra\u2010Intestinal Features in Patients With Systemic Sclerosis"],"prefix":"10.1002","volume":"75","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3774-6087","authenticated-orcid":false,"given":"Ana E.","family":"Tucker","sequence":"first","affiliation":[{"name":"Medical University of South Carolina  Charleston"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jamie","family":"Perin","sequence":"additional","affiliation":[{"name":"Johns Hopkins Bloomberg 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