Interstitial lung disease in polymyositis/dermatomyositis

Comprehensive screening can disclose evidence of interstitial lung disease (ILD) in about 40 % of patients with polymyositis/dermatomyositis (PM/DM). In most cases this is a non-specific interstitial pneumonia, a histopathological type characterized by a substantial initial inflammatory component. Autoantibody testing, spirometry, including the measurement of the the CO diffusion capacity and high-resolution computed tomography (HRCT) are central to making the diagnosis during the early inflammatory stages of ILD. This article outlines the use of clinical, HRCT and bronchoalveolar lavage (BAL) findings for prognostication and gives an overview of current treatment options.