Idiopathic inflammatory myopathies and the lung
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 m...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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European Respiratory Society
2015-06-01
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| Series: | European Respiratory Review |
| Online Access: | http://err.ersjournals.com/content/24/136/216.full |
| _version_ | 1819076455019053056 |
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| author | Jean-Christophe Lega Quitterie Reynaud Alexandre Belot Nicole Fabien Isabelle Durieu Vincent Cottin |
| author_facet | Jean-Christophe Lega Quitterie Reynaud Alexandre Belot Nicole Fabien Isabelle Durieu Vincent Cottin |
| author_sort | Jean-Christophe Lega |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-21T18:41:34Z |
| format | Article |
| id | doaj.art-02e1cec6af10427497377a84f6b75f02 |
| institution | Directory Open Access Journal |
| issn | 0905-9180 1600-0617 |
| language | English |
| last_indexed | 2024-12-21T18:41:34Z |
| publishDate | 2015-06-01 |
| publisher | European Respiratory Society |
| record_format | Article |
| series | European Respiratory Review |
| spelling | doaj.art-02e1cec6af10427497377a84f6b75f022022-12-21T18:54:00ZengEuropean Respiratory SocietyEuropean Respiratory Review0905-91801600-06172015-06-012413621623810.1183/16000617.0000201502015Idiopathic inflammatory myopathies and the lungJean-Christophe Lega0Quitterie Reynaud1Alexandre Belot2Nicole Fabien3Isabelle Durieu4Vincent Cottin5 Dept of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France Dept of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France Dept of Pediatric Rheumatology, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France Dept of Immunology, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Lyon, France Dept of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France 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 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.http://err.ersjournals.com/content/24/136/216.full |
| spellingShingle | Jean-Christophe Lega Quitterie Reynaud Alexandre Belot Nicole Fabien Isabelle Durieu Vincent Cottin Idiopathic inflammatory myopathies and the lung European Respiratory Review |
| title | Idiopathic inflammatory myopathies and the lung |
| title_full | Idiopathic inflammatory myopathies and the lung |
| title_fullStr | Idiopathic inflammatory myopathies and the lung |
| title_full_unstemmed | Idiopathic inflammatory myopathies and the lung |
| title_short | Idiopathic inflammatory myopathies and the lung |
| title_sort | idiopathic inflammatory myopathies and the lung |
| url | http://err.ersjournals.com/content/24/136/216.full |
| work_keys_str_mv | AT jeanchristophelega idiopathicinflammatorymyopathiesandthelung AT quitteriereynaud idiopathicinflammatorymyopathiesandthelung AT alexandrebelot idiopathicinflammatorymyopathiesandthelung AT nicolefabien idiopathicinflammatorymyopathiesandthelung AT isabelledurieu idiopathicinflammatorymyopathiesandthelung AT vincentcottin idiopathicinflammatorymyopathiesandthelung |