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Myositis-associated interstitial lung disease: a comprehensive approach to diagnosis and management

1, 2


  1. Department of Pulmonary and Critical Care Medicine, Massachusetts General Hospital, and Harvard Medical School, Boston, USA.
  2. Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

2022 Vol.40, N°2
PI 0373, PF 0383

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PMID: 33769263 [PubMed]

Received: 21/12/2020
Accepted : 08/02/2021
In Press: 25/03/2021
Published: 25/02/2022


Interstitial lung disease (ILD) frequently complicates the inflammatory myopathies and at times is the most prominent clinical feature. Over the years, there has been a growing recognition for the strong association between seropositivity of several myositis-specific antibodies (MSAs) and lung involvement. Growing literature suggests that individual MSAs may influence the risk of developing ILD and are associated with pulmonary disease severity and various clinical sub-phenotypes. The presence of ILD in patients with myositis correlates with increased morbidity and mortality. As such, it presents a unique treatment challenge for both the rheumatology and pulmonary communities and requires a multidisciplinary approach to management. This review will discuss the role of serologies and invasive and non-invasive testing modalities utilised to diagnose and monitor patients with myositis-ILD. Current studies pertaining to the wide array of immunomodulatory therapies utilised in cases of progressive disease are also highlighted in detail.


Rheumatology Article