{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T22:54:14Z","timestamp":1793919254555,"version":"4.8.0"},"reference-count":15,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2017,10,27]],"date-time":"2017-10-27T00:00:00Z","timestamp":1509062400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100007880","name":"Johns Hopkins University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007880","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["AR\u2010053503"],"award-info":[{"award-number":["AR\u2010053503"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","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\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis Care &amp; Research"],"published-print":{"date-parts":[[2017,11]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To determine if a unique subtype of scleroderma muscle disease exists by comparing the clinical features of systemic sclerosis (<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c; scleroderma) patients with predominant fibrosis on muscle biopsy to those with inflammatory muscle histopathology.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This retrospective, cross\u2010sectional study included <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients with muscle weakness and an available muscle biopsy. Biopsies with fibrosis but without inflammation\/necrosis were designated as \u201cfibrosing myopathy,\u201d and those with inflammation and\/or necrosis were assigned a category of \u201cinflammatory myopathy.\u201d Clinical data, including features of <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c, serum creatine kinase (<jats:styled-content style=\"fixed-case\">CK<\/jats:styled-content>) levels, electromyography, autoantibody profile, and survival, were compared between the 2 groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The study population consisted of 37 weak <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients, 8 with fibrosing myopathy and 29 with inflammatory myopathy. Compared to those with inflammatory myopathy, patients with fibrosing myopathy were more likely to have diffuse <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c skin subtype (87% versus 62%; <jats:italic>P<\/jats:italic> = 0.18), African American race (62.5% versus 37.9%; <jats:italic>P<\/jats:italic> = 0.20), and a lower mean \u00b1 <jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content> forced vital capacity (55.5 \u00b1 31.9 versus 66.4 \u00b1 17.6; <jats:italic>P<\/jats:italic> = 0.23). They also had lower mean \u00b1 <jats:styled-content style=\"fixed-case\">SD CK<\/jats:styled-content> values (516 \u00b1 391 versus 2,477 \u00b1 3,511 <jats:styled-content style=\"fixed-case\">IU<\/jats:styled-content>\/liter; <jats:italic>P<\/jats:italic> = 0.007) and lower aldolase values (13.8 \u00b1 4.7 versus 27.3 \u00b1 4.7; <jats:italic>P<\/jats:italic> = 0.01). Patients with fibrosing myopathy had a significantly higher mortality (5 of 8 [62.5%] versus 4 of 29 [14.3%]; <jats:italic>P<\/jats:italic> = 0.005).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Fibrosing myopathy is a unique histologic subtype of muscle disease among weak patients with <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c and is associated with significantly worse mortality compared to those with inflammation and\/or necrosis on muscle biopsy.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.23291","type":"journal-article","created":{"date-parts":[[2017,5,23]],"date-time":"2017-05-23T21:11:31Z","timestamp":1495573891000},"page":"1764-1770","source":"Crossref","is-referenced-by-count":46,"title":["Association of Fibrosing Myopathy in Systemic Sclerosis and Higher Mortality"],"prefix":"10.1002","volume":"69","author":[{"given":"Julie J.","family":"Paik","sequence":"first","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":"Ami A.","family":"Shah","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea M.","family":"Corse","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"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"}]},{"given":"Laura K.","family":"Hummers","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew L.","family":"Mammen","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore, Maryland, and National Institute of Arthritis and Musculoskeletal and Skin Diseases\/NIH Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,10,27]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.22870"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.121380"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.3109\/03009742.2013.868512"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780110405"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780210111"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.22620"},{"key":"e_1_2_7_8_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_9_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.159.1.9712108"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1164\/arrd.1987.135.4.805"},{"key":"e_1_2_7_11_1","first-page":"17","volume-title":"Evaluation of patients with myopathy","author":"Griggs R","year":"1995"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2007-971997"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2004.02.006"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.21992"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-8703(93)90075-K"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.24409"}],"container-title":["Arthritis Care &amp; Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Facr.23291","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr.23291","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T15:14:56Z","timestamp":1759418096000},"score":1,"resource":{"primary":{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/10.1002\/acr.23291"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,10,27]]},"references-count":15,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2017,11]]}},"alternative-id":["10.1002\/acr.23291"],"URL":"https:\/\/doi.org\/10.1002\/acr.23291","archive":["Portico"],"relation":{},"ISSN":["2151-464X","2151-4658"],"issn-type":[{"value":"2151-464X","type":"print"},{"value":"2151-4658","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,10,27]]}}}