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

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.