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Except for inclusion body myositis, these extrapulmonary disorders are associated with the general and visceral involvement frequently found in other CTDs including fever, Raynaud's phenomenon, arthralgia, nonspecific cutaneous modifications and ILD, for which the prevalence is estimated to be up to 65%. Substantial heterogeneity exists within the spectrum of IIMs, and each condition is associated with various frequencies and subtypes of pulmonary involvement. This heterogeneity is partly related to the presence of various autoantibodies encompassing anti-synthetase, anti-MDA5 and anti-PM\/Scl. ILD is present in all subsets of IIM including juvenile myositis, but is more frequent in dermatomyositis and overlap myositis. IIM can also be associated with other presentations of respiratory involvement, namely pulmonary arterial hypertension, pleural disease, infections, drug-induced toxicity, malignancy and respiratory muscle weakness. Here, we critically review the current knowledge about adult and juvenile myositis-associated lung disease with a detailed description of therapeutics for chronic and rapidly progressive ILD.<\/jats:p>","DOI":"10.1183\/16000617.00002015","type":"journal-article","created":{"date-parts":[[2015,6,1]],"date-time":"2015-06-01T00:14:30Z","timestamp":1433117670000},"page":"216-238","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":176,"title":["Idiopathic inflammatory myopathies and the lung"],"prefix":"10.1183","volume":"24","author":[{"given":"Jean-Christophe","family":"Lega","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Quitterie","family":"Reynaud","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre","family":"Belot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Fabien","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Durieu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Cottin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2015,5,31]]},"reference":[{"key":"2024102403153503000_24.136.216.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197502132920706"},{"key":"2024102403153503000_24.136.216.2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197502202920807"},{"key":"2024102403153503000_24.136.216.3","doi-asserted-by":"publisher","DOI":"10.1002\/art.24372"},{"key":"2024102403153503000_24.136.216.4","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1136\/annrheumdis-2012-201800","article-title":"Patients with non-Jo-1 anti-tRNA-synthetase autoantibodies have worse survival than Jo-1 positive patients","volume":"73","author":"Aggarwal","year":"2013","journal-title":"Ann Rheum Dis"},{"key":"2024102403153503000_24.136.216.5","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00156312"},{"key":"2024102403153503000_24.136.216.6","doi-asserted-by":"crossref","unstructured":"Dobloug C , Garen T , Bitter H , et al. 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