Interstitial Lung Disease in Polymyositis and Dermatomyositis

Kathryn Long; Sonye K Danoff

doi:10.1016/j.ccm.2019.05.004

Interstitial Lung Disease in Polymyositis and Dermatomyositis

Clin Chest Med. 2019 Sep;40(3):561-572. doi: 10.1016/j.ccm.2019.05.004.

Authors

Kathryn Long¹, Sonye K Danoff²

Affiliations

¹ Johns Hopkins Hospital, 600 N Wolfe Street, Osler 292-A, Baltimore, MD 21287, USA.
² Department of Medicine, Division of Pulmonary and Critical Care Medicine, Johns Hopkins School of Medicine, 1830 East Monument Street, 5th Floor, Baltimore, MD 212015, USA. Electronic address: sdanoff@jhmi.edu.

PMID: 31376891
DOI: 10.1016/j.ccm.2019.05.004

Abstract

The idiopathic inflammatory myopathies (IIMs), including polymyositis (PM) and dermatomyositis (DM), are autoimmune connective tissue diseases with variable degrees of muscle inflammation and systemic involvement. Interstitial lung disease (ILD) is a common complication of the IIMs and is associated with increased mortality. Many patients with PM/DM have myositis-specific and myositis-associated antibodies (MSA/MAAs) that result in distinct clinical phenotypes. Among these MSAs, anti-aminoacyl-tRNA antibodies and anti-melanoma differentiation factor 5 antibodies have high rates of ILD. Corticosteroids are the mainstay of treatment, although the addition of other immunosuppressive therapy is typically necessary to achieve disease control.

Keywords: Dermatomyositis; Interstitial lung disease; Myositis; Polymyositis.

Publication types

Review

MeSH terms

Adult
Dermatomyositis / etiology*
Dermatomyositis / pathology
Female
Humans
Lung Diseases, Interstitial / complications*
Lung Diseases, Interstitial / pathology
Male
Middle Aged
Polymyositis / etiology*
Polymyositis / pathology