{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T15:21:35Z","timestamp":1787757695436,"version":"build-2784847793"},"reference-count":27,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T00:00:00Z","timestamp":1774310400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T00:00:00Z","timestamp":1774310400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis Care &amp;amp; Research"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>The objective of this study was to describe the longitudinal disease course and pulmonary outcomes of North American patients with melanoma differentiation\u2013associated protein 5 (MDA5) antibody\u2013associated dermatomyositis (DM).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Thirty patients with MDA5 antibody\u2013associated DM were identified in a single\u2010center longitudinal cohort of 352 patients with idiopathic inflammatory myopathies. Longitudinal assessments of patient clinical and laboratory disease characteristics, pulmonary function tests (PFT), and high\u2010resolution computed tomography chest scans were conducted.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Eighty percent (n\u00a0=\u00a024\/30) of patients with MDA5 antibody\u2013associated DM had interstitial lung disease (ILD). The overall mortality was low (2\/24 at a mean \u00b1 SD follow\u2010up of 4.0\u2009\u00b1\u20090.8 years). At this follow\u2010up, patients were receiving 3.1\u2009\u00b1\u20091.3 therapies, including 79% receiving intravenous Ig (IVIg), 58% receiving rituximab, 67% receiving mycophenolate, and 63% receiving glucocorticoids. In 18 of 22 surviving patients with ILD who had two\u2010year longitudinal follow\u2010up available at 1.8\u2009\u00b1\u20090.6 years, improvements of 16% and 17% predicted forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DL\n                      <jats:sc>co<\/jats:sc>\n                      ) were noted. In 10 of 18 patients with additional long\u2010term follow\u2010up available (mean \u00b1 SD 6.8\u2009\u00b1\u20093.4 years), improvements of 24% and 20% predicted FVC and DL\n                      <jats:sc>co<\/jats:sc>\n                      were noted. MDA5 antibody and interleukin\u201015 (IL\u201015) levels and paraoxonase 1 (PON1) enzyme activity correlated significantly with disease activity at baseline and longitudinally.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In a North American MDA5 antibody\u2013associated DM\u2010ILD cohort treated with aggressive combination immunomodulatory therapy including predominantly mycophenolate, IVIg, and rituximab, disease mortality was low and lung function improved markedly. IL\u201015, PON1, and MDA5 antibody titers warrant further investigation as disease activity biomarkers in this high\u2010risk population.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/acr.80004","type":"journal-article","created":{"date-parts":[[2026,1,20]],"date-time":"2026-01-20T12:56:53Z","timestamp":1768913813000},"page":"1112-1122","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Marked Long\u2010Term Improvement in Lung Function in Melanoma Differentiation\u2013Associated Protein 5 Antibody\u2013Positive Dermatomyositis Patients: Experience of a Single\u2010Center Longitudinal Cohort in North America"],"prefix":"10.1002","volume":"78","author":[{"given":"Jenice X.","family":"Cheah","sequence":"first","affiliation":[{"name":"University of California  Los Angeles"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7216-7219","authenticated-orcid":false,"given":"Sangmee S.","family":"Bae","sequence":"additional","affiliation":[{"name":"University of California  Los Angeles"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiffany","family":"De Leon","sequence":"additional","affiliation":[{"name":"University of California  Los Angeles"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuna","family":"Lee","sequence":"additional","affiliation":[{"name":"University of California  Los Angeles"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rong","family":"Guo","sequence":"additional","affiliation":[{"name":"University of California  Los Angeles"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Elashoff","sequence":"additional","affiliation":[{"name":"University of California  Los Angeles"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Wang","sequence":"additional","affiliation":[{"name":"University of California  Los Angeles"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ani","family":"Shahbazian","sequence":"additional","affiliation":[{"name":"University of California  Los Angeles"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina","family":"Charles\u2010Schoeman","sequence":"additional","affiliation":[{"name":"University of California  Los Angeles"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,3,24]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/BOR.0000000000000908"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-199111000-00002"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11926-002-0089-7"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00296-021-04819-1"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.21992"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2010.09.016"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2015.07.004"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2023.01.031"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCRESAHA.123.321563"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keaa273"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keab795"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.40320"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-020-74016-w"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38118"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.41105"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.2023-0371"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(16)30152-7"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.42861"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keab928"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.3109\/s10165-012-0663-4"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-12-3603"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.8178155"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.93.7.2897"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12338641"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/joim.13154"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2018.06.012"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2017.12.010"}],"container-title":["Arthritis Care &amp; 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