PT - JOURNAL ARTICLE AU - Lega, Jean-Christophe AU - Reynaud, Quitterie AU - Belot, Alexandre AU - Fabien, Nicole AU - Durieu, Isabelle AU - Cottin, Vincent TI - Idiopathic inflammatory myopathies and the lung AID - 10.1183/16000617.00002015 DP - 2015 Jun 01 TA - European Respiratory Review PG - 216--238 VI - 24 IP - 136 4099 - https://publications.ersnet.org//content/24/136/216.short 4100 - https://publications.ersnet.org//content/24/136/216.full SO - EUROPEAN RESPIRATORY REVIEW2015 Jun 01; 24 AB - Idiopathic inflammatory myositis (IIM) is a group of rare connective tissue diseases (CTDs) characterised by muscular and extramuscular signs, in which lung involvement is a challenging issue. Interstitial lung disease (ILD) is the hallmark of pulmonary involvement in IIM, and causes morbidity and mortality, resulting in an estimated excess mortality of 50% in some series. 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. Interstitial lung disease is a leading cause of morbidity in dermatomyositis/polymyositis http://ow.ly/M1wB8