Interstitial Lung Disease in Polymyositis and Dermatomyositis

The idiopathic inflammatory myopathies (Ws), including polymyositis (PM) and dermatomyositis (DM), are autoimmune connective tissue diseases with variable degrees of muscle inflammation and systemic involvement. Interstitial lung disease (ILD) is a common complication of the Ws and is associated with increased mortality. Many patients with PM/DM have myositis-specific and myositis-associated antibodies (MSA/MAAs) that result in distinct clinical phenotypes. Among these MSAs, anti-aminoacyl-tRNA antibodies and anti-melanoma differentiation factor 5 antibodies have high rates of ILD. Corticosteroids are the mainstay of treatment, although the addition of other immunosuppressive therapy is typically necessary to achieve disease control.