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Review
. 2015 Jun;24(136):216-38.
doi: 10.1183/16000617.00002015.

Idiopathic inflammatory myopathies and the lung

Jean-Christophe Lega  1 , Quitterie Reynaud  2 , Alexandre Belot  3 , Nicole Fabien  4 , Isabelle Durieu  2 , Vincent Cottin  5
Affiliations
Review

Idiopathic inflammatory myopathies and the lung

Jean-Christophe Lega et al. Eur Respir Rev. 2015 Jun.

Erratum in

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.

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Conflict of interest statement

Conflict of interest: Disclosures can be found alongside the online version of this article at err.ersjournals.com

Figures

FIGURE 1
FIGURE 1
Raynaud's phenomenon in a patient positive for anti-PL7 antibodies.
FIGURE 2
FIGURE 2
Clinical features associated with anti-MDA5 autoantibodies include severe cutaneous ulcers of a, b) the fingers and c) the dorsal side of the elbow.
FIGURE 3
FIGURE 3
Mechanic's hands in a patient with anti-MAD5 positive dermatomyositis. Image courtesy of Sébastien Debarbieux (Dept of Dermatology, Centre Hospitalier Lyon Sud, Lyon, France).
FIGURE 4
FIGURE 4
High-resolution computed tomography from a patient with anti-Jo1 positive polymyositis showing basilar predominate reticulation and ground-glass opacity without honeycombing, in a pattern suggestive of nonspecific interstitial pneumonia.
FIGURE 5
FIGURE 5
High-resolution computed tomography from a patient with polymyositis showing honeycombing in a pattern consistent with usual interstitial pneumonia.
See this image and copyright information in PMC

References

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