New paradigm in the treatment of myositis-associated interstitial lung disease

Interstitial lung disease (ILD) is the leading cause of mortality in idiopathic inflammatory myopathies or myositis. Clinical characteristics, including the course of ILD, rate of progression, radiological and pathohistological morphologies, extent and distribution of inflammation and fibrosis, responses to treatment, recurrence rate, and prognosis, are highly variable among myositis patients. A standard practice for ILD management in myositis patients has not yet been established.

Recent studies have demonstrated the stratification of patients with myositis-associated ILD into more homogeneous groups based on the disease behavior and myositis-specific autoantibody (MSA) profile, leading to better prognoses and prevention of the burden of organ damage. This review introduces a new paradigm in the management of myositis-associated ILD based on research findings from relevant literature selected by a search of PubMed as of January 2023, as well as expert opinions.

Managing strategies for myositis-associated ILD are being established to stratify patients based on the severity of ILD and the prediction of prognosis based on the disease behavior and MSA profile. The development of a precision medicine treatment approach will provide benefits to all relevant communities.