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Interstitial Lung Disease Associated With the Idiopathic Inflammatory Myopathies: What Progress Has Been Made in the Past 35 Years?

Geoffrey R. Connors, MD; Lisa Christopher-Stine, MD; Chester V. Oddis, MD; Sonye K. Danoff, MD, PhD, FCCP
Author and Funding Information

From the Department of Medicine, Division of Pulmonary and Critical Care Medicine (Drs Connors and Danoff), and Division of Rheumatology (Dr Christopher-Stine), Johns Hopkins University School of Medicine, Baltimore, MD; and the Department of Medicine, Division of Rheumatology (Dr Oddis), University of Pittsburgh Medical Center, Pittsburgh, PA.

Correspondence to: Sonye K. Danoff, MD, PhD, FCCP; Pulmonary and Critical Care Medicine, 1830 E Monument St, 5th Floor, Baltimore, MD 21210; e-mail: sdanoff@jhmi.edu


Funding/Support: This work was supported by funding from the Lisa Sandler Spaeth Fund to S. K. D.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2010 American College of Chest Physicians


Chest. 2010;138(6):1464-1474. doi:10.1378/chest.10-0180
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Extract

The idiopathic inflammatory myopathies (IIMs) affect more than 60,000 individuals in the United States and an estimated 770,000 worldwide.1 Although widely considered a disease confined to muscle, interstitial lung disease (ILD) remains one of the greatest contributors to morbidity and mortality in IIM, resulting in an estimated excess mortality of 40%.2 Myositis-associated interstitial lung disease (MA-ILD) may occur in the context of virtually all of the IIIM and yet remains poorly understood. Since the first reviews and comprehensive descriptions nearly 35 years ago by Frazier and Miller3 and Schwarz et al,4 many case series and a few prospective studies have addressed the pathogenesis of, and therapy for, this condition. Despite these efforts, ILD is independently associated with a poor quality of life for patients with myositis even when the response to the myopathic, arthritic, and dermatologic manifestations is favorable.

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