Semin Respir Crit Care Med 2014; 35(02): 239-248
DOI: 10.1055/s-0034-1371528
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Pulmonary Manifestations of Polymyositis/Dermatomyositis

Robert W. Hallowell
1   Division of Pulmonary and Critical Medicine, John Hopkins School of Medicine, Baltimore, Maryland
,
Dana P. Ascherman
2   Division of Rheumatology, University of Miami Health System, Miami, Florida
,
Sonye K. Danoff
1   Division of Pulmonary and Critical Medicine, John Hopkins School of Medicine, Baltimore, Maryland
3   Johns Hopkins Myositis Center, John Hopkins School of Medicine, Baltimore, Maryland
› Author Affiliations
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Publication History

Publication Date:
25 March 2014 (online)

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Abstract

The idiopathic inflammatory myopathies are a group of connective tissue diseases marked by varying degrees of muscle inflammation and clinical involvement of multiple organs, most notably, the lung. Pulmonary manifestations consist primarily of interstitial lung disease (ILD), which is associated with significant morbidity and mortality in myositis patients. Several myositis-specific antibodies have been discovered, as well as antibodies targeting various aminoacyl-tRNA synthetase enzymes. These antibodies are associated with various clinical features and a risk for developing ILD, and their presence carries a prognostic value in myositis patients. Steroids remain the first-line treatment for myositis-associated ILD and the antisynthetase syndrome, though other traditional immunosuppressants have demonstrated efficacy in numerous studies. While a majority of patients experience either stabilization or improvement in lung imaging and function, fatal progression is still reported in a significant number of cases. Further research is needed to develop more effective and targeted therapies.