Interstitial lung disease associated with the idiopathic inflammatory myopathies and the antisynthetase syndrome: Recent advances

Robert W. Hallowell, Sonye K. Danoff

Research output: Contribution to journalReview articlepeer-review

33 Scopus citations


Purpose of review: To highlight recent advances in understanding the clinical spectrum, pathogenesis, and treatment of interstitial lung disease associated with inflammatory myositis and the antisynthetase syndrome. Recent findings: In recent years, serologic tests to identify the less common antisynthetase antibodies and the anti-MDA-5 antibody have become commercially available. As a result, several large, retrospective analyses have illustrated both the pulmonary and non-pulmonary features associated with the antisynthetase syndrome and myositis-related interstitial lung disease. Notably, there is now a better appreciation for the heterogeneity of these syndromes and the prognostic value in accurately identifying the associated autoantibodies. Human cytokine profiling and murine models of muscle inflammation suggest that tRNA synthetases themselves may act to trigger an initial innate immune response, thus offering new insights into the pathophysiology of these diseases. Finally, although randomized clinical trials in patients with myositis-associated interstitial lung disease have not occurred, new observational studies suggest that cyclosporine, tacrolimus, and rituximab may be effective treatment options. Summary: Recent research has provided a better understanding of the phenotype and prognosis that define interstitial lung disease in the setting of myositis and the antisynthetase syndrome. Although several therapeutic agents demonstrate promise, randomized trials are needed in order to establish the best clinical approach in these patients. Furthermore, additional research into the pathophysiology of this disease will be necessary to develop newer, more targeted therapeutics.

Original languageEnglish (US)
Pages (from-to)684-689
Number of pages6
JournalCurrent opinion in rheumatology
Issue number6
StatePublished - Jan 1 2014


  • Antisynthetase syndrome
  • Idiopathic inflammatory myopathy
  • Interstitial lung disease
  • Myositis

ASJC Scopus subject areas

  • Rheumatology


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