{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T07:36:43Z","timestamp":1780472203193,"version":"3.54.1"},"reference-count":15,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2019,10,29]],"date-time":"2019-10-29T00:00:00Z","timestamp":1572307200000},"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 Care &amp; Research"],"published-print":{"date-parts":[[2019,11]]},"abstract":"Objective<\/jats:title>Juvenile systemic sclerosis (SS<\/jats:styled-content>c) is a disabling autoimmune condition that affects multiple organs in addition to skin, notably the gastrointestinal and pulmonary systems. The relationship between esophageal abnormalities and pulmonary disease in juvenile SS<\/jats:styled-content>c is not well understood. We describe associations between radiologic esophageal abnormalities and pulmonary function.<\/jats:p><\/jats:sec>Methods<\/jats:title>Clinical and radiographic data of children ages >18 years who fulfilled the 2007 Pediatric Rheumatology Provisional Classification Criteria for juvenile SS<\/jats:styled-content>c between 1994 and 2016 were reviewed. Fluoroscopic upper gastrointestinal (UGI<\/jats:styled-content>) studies, high\u2010resolution computed tomography (HRCT<\/jats:styled-content>), and pulmonary function tests (PFT<\/jats:styled-content>s) within 12 months of presentation to Seattle Children's Hospital were extracted.<\/jats:p><\/jats:sec>Results<\/jats:title>Twenty\u2010one children with juvenile SS<\/jats:styled-content>c (67% female, ages 8\u201317 years) were studied. Esophageal abnormalities, defined as abnormal esophageal peristalsis and\/or bolus clearance, were found in 12 patients. Abnormal esophagus on UGI<\/jats:styled-content> tests was not associated with gastrointestinal or pulmonary symptoms, disease duration, use of medications (proton pump inhibitor or immunosuppressant), or specific autoantibodies. Compared with patients with a normal esophagus on UGI<\/jats:styled-content> tests, children with an abnormal esophagus had decreased PFT<\/jats:styled-content>s: mean forced expiratory volume in 1 second 96% versus 78% (P<\/jats:italic> = 0.03), forced vital capacity 94% versus 76% (P<\/jats:italic> = 0.02), and vital capacity 95% versus 76% (P<\/jats:italic> = 0.02). Children with an abnormal esophagus on UGI<\/jats:styled-content> tests had a larger mean esophageal diameter on HRCT<\/jats:styled-content> (14.6 mm compared to 8.5 mm; P<\/jats:italic> < 0.01).<\/jats:p><\/jats:sec>Conclusion<\/jats:title>There was an association between esophageal and pulmonary disease in children with juvenile SS<\/jats:styled-content>c. Esophageal findings on UGI<\/jats:styled-content> tests or HRCT<\/jats:styled-content>, despite lack of symptoms, should raise concern for esophageal dysfunction and prompt heightened surveillance for concurrent lung disease.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.23778","type":"journal-article","created":{"date-parts":[[2018,10,8]],"date-time":"2018-10-08T05:53:26Z","timestamp":1538978006000},"page":"1444-1449","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Relationship Between Esophageal Abnormalities on Fluoroscopic Esophagram and Pulmonary Function Testing in Juvenile Systemic Sclerosis"],"prefix":"10.1002","volume":"71","author":[{"given":"Lusine","family":"Ambartsumyan","sequence":"first","affiliation":[{"name":"Seattle Children's Hospital University of Washington Seattle"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hengqi 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