{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:50:16Z","timestamp":1790729416482,"version":"4.1.0"},"reference-count":40,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2026,1,29]],"date-time":"2026-01-29T00:00:00Z","timestamp":1769644800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,1,29]],"date-time":"2026-01-29T00:00:00Z","timestamp":1769644800000},"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":["ACR Open Rheumatology"],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Interstitial lung disease (ILD) is common in idiopathic inflammatory myositis (IIM), particularly in antisynthetase syndrome (ASyS), antimelanoma differentiation\u2010associated protein 5 (anti\u2010MDA5) syndrome, and scleromyositis. ILD can progress despite resolution of extrapulmonary symptoms, termed postmyopathic progressive pulmonary fibrosis (PmPPF). We outlined ILD trajectories in these subgroups of IIM, focusing on PmPPF.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A retrospective review of patients with IIM (ASyS, anti\u2010MDA5+ dermatomyositis [DM], scleromyositis) from a British Columbia cohort (2019\u20132024) assessed demographics, auto\u2010antibodies, treatments, and ILD progression.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Among 111 patients with ASyS, anti\u2010MDA5+ DM, and scleromyositis (median age 51 years, follow\u2010up 35.5 months), ILD prevalence was highest in ASyS (87.5%), followed by anti\u2010MDA5+ DM (84.6%), and scleromyositis (65.5%). PmPPF occurred in 13.5% of the cohort, predominantly in anti\u2010MDA5+ DM (23.1%) and ASyS (16.1%) but not in scleromyositis. Patients with PmPPF had higher rates of active disease (46.7% vs 9.3%), rapidly progressive ILD (33.3% vs 5.3%), and lung transplantation (20% vs 1.3%). Anti\u2010MDA5+ DM exhibited the highest remission rate (88.5%) but also the highest transplant requirement (11.5%). Organizing pneumonia (OP)\/nonspecific interstitial pneumonia (NSIP) overlap on high\u2010resolution computed tomography was more common in PmPPF (33.3% vs 9.3%). PmPPF showed a nonsignificant trend toward higher mortality compared to non\u2010PmPPF (20% vs 4%). Rituximab use was greater in PmPPF (73.3% vs 37.3%).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This study highlights distinct ILD patterns across three idiopathic inflammatory myopathy subtypes, with PmPPF occurring more frequently in ASyS and anti\u2010MDA5+ DM but not in scleromyositis. The persistence of ILD progression independent of extrapulmonary disease activity underscores the importance of ongoing pulmonary monitoring and multidisciplinary management.<\/jats:p>\n                    <jats:p>\n                      <jats:boxed-text content-type=\"graphic\" position=\"anchor\">\n                        <jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/acr290010-gra-0001-m.png\">\n                          <jats:alt-text>image<\/jats:alt-text>\n                        <\/jats:graphic>\n                      <\/jats:boxed-text>\n                    <\/jats:p>\n                  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