title: Idiopathic inflammatory myopathies and the lung.
abstract: 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.
authors.affiliation: Dept of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France UMR 5558, Laboratoire de Biométrie et Biologie Evolutive, CNRS, Claude Bernard University Lyon 1, University of Lyon, Lyon, France jean-christophe.lega@chu-lyon.fr.
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Lega
authors.affiliation: Dept of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France.
authors.forename: Quitterie
authors.initials: Q
authors.lastname: Reynaud
authors.affiliation: Dept of Pediatric Rheumatology, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France.
authors.forename: Alexandre
authors.initials: A
authors.lastname: Belot
authors.affiliation: Dept of Immunology, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Lyon, France.
authors.forename: Nicole
authors.initials: N
authors.lastname: Fabien
authors.affiliation: Dept of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Durieu
authors.affiliation: National Reference Centre for Rare Pulmonary Diseases, Dept of Respiratory Medicine, Louis Pradel Hospital, Hospices Civils de Lyon, UMR 754, Claude Bernard University Lyon 1, University of Lyon, Lyon, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D015897:Comorbidity; D018450:Disease Progression; D004359:Drug Therapy, Combination; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008168:Lung / Q000187:drug effects / Q000276:immunology / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000503:physiopathology / Q000628:therapy*; D009220:Myositis / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D011237:Predictive Value of Tests; D011379:Prognosis; D012307:Risk Factors; D013997:Time Factors
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D007166:Immunosuppressive Agents