Management of Lung Manifestations in Idiopathic Inflammatory Myopathies

Pulmonary complications are a major source of morbidity and mortality in patients with idiopathic inflammatory myopathies (IIM). Interstitial lung disease is the most common significant pulmonary manifestation of IIM, and intensive treatment with immunomodulatory medications may be necessary. Other common pulmonary complications of IIM include pneumonia, pneumomediastinum, pulmonary hypertension, aspiration, respiratory muscle weakness, and increased risk for lung cancer. The workup of shortness of breath in this population requires a comprehensive multidisciplinary assessment by rheumatology and pulmonology, with testing that may include pulmonary function tests (PFTs), computed tomography scan, and echocardiography.