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      Idiopathic inflammatory myopathies and the lung

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          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.

          Abstract

          Interstitial lung disease is a leading cause of morbidity in dermatomyositis/polymyositis http://ow.ly/M1wB8

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          Most cited references176

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          Polymyositis and dermatomyositis (first of two parts).

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            Activated STING in a vascular and pulmonary syndrome.

            The study of autoinflammatory diseases has uncovered mechanisms underlying cytokine dysregulation and inflammation.
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              119th ENMC international workshop: trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10-12 October 2003, Naarden, The Netherlands.

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                Author and article information

                Journal
                Eur Respir Rev
                Eur Respir Rev
                ERR
                errev
                European Respiratory Review
                European Respiratory Society
                0905-9180
                1600-0617
                June 2015
                : 24
                : 136
                : 216-238
                Affiliations
                [1 ]Dept of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France
                [2 ]UMR 5558, Laboratoire de Biométrie et Biologie Evolutive, CNRS, Claude Bernard University Lyon 1, University of Lyon, Lyon, France
                [3 ]Dept of Pediatric Rheumatology, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France
                [4 ]Dept of Immunology, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Lyon, France
                [5 ]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
                Author notes
                Jean-Christophe Lega, Dept of Internal and Vascular Medicine, Hospices Civils de Lyon, Centre Hospitalier Lyon Sud, 69495 Pierre-Bénite, France. E-mail: jean-christophe.lega@ 123456chu-lyon.fr
                Article
                ERR-0020-2015
                10.1183/16000617.00002015
                9487811
                26028634
                a051fb48-ab42-46a8-9b6d-c0ec5b0b7295
                Copyright ©ERS 2015.

                ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

                History
                : 11 March 2015
                : 22 April 2015
                Categories
                European Respiratory Update
                13

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