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Treatment of Interstitial Lung Disease Associated With Myositis and the Anti-Synthetase Syndrome

  • Other CTD: Inflammatory Myopathies and Sjögren's (P Basharat, Section Editor)
  • Published:
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Abstract

Purpose of review

The idiopathic inflammatory myopathies, including the anti-synthetase syndrome, are frequently complicated by interstitial lung disease (ILD). ILD in these patients can range from indolent to fulminant, and its presence is associated with increased morbidity and mortality. This review will focus on the most recent evidence guiding the management of myositis-associated ILD.

Recent findings

Treatment of ILD typically involves the use of corticosteroids in combination with one or more immunosuppressive agents. Evidence supporting the use of each agent is limited to retrospective studies and case series, and there may be a benefit to starting more intensive therapy at the time of ILD diagnosis. Response to therapy is monitored by serial pulmonary function testing, as well as functional studies assessing O2 requirements with ambulation. A majority of patients demonstrate stabilization or improvement in pulmonary function with therapy; however, a minority worsens despite treatment. In a select group of these patients who fail to respond to conventional therapy, lung transplantation may be an option.

Summary

Despite limited supporting data, a combination of corticosteroids and various immunosuppressive agents remains the first-line treatment of myositis-associated ILD. Outcomes are variable, and randomized trials are needed to determine the most effective combination of these drugs.

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Authors and Affiliations

  1. Beth Israel Deaconess Medical Center, Boston, MA, USA

    Robert W. Hallowell MD

  2. Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Sonye K. Danoff MD, PhD

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  1. Robert W. Hallowell MD
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This article is part of the Topical Collection on Other CTD: Inflammatory Myopathies and Sjögren’s

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Hallowell, R.W., Danoff, S.K. Treatment of Interstitial Lung Disease Associated With Myositis and the Anti-Synthetase Syndrome. Curr Treat Options in Rheum 4, 316–328 (2018). https://doi.org/10.1007/s40674-018-0111-5

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