Connective tissue diseases (CTDs) can affect the lungs through diseases of the chest wall, pleura, vasculature, airways, and parenchyma. Interstitial lung disease (ILD) is a common complication of CTD associated with increased morbidity and mortality. This article describes the evaluation of respiratory impairment in patients with CTD and summarizes the evidence that guides diagnosis and management of CTD-ILD. Patients with CTD with suspected ILD should undergo clinical, physiologic, and radiologic studies to evaluate for the presence of ILD, and these results should be integrated in a multidisciplinary setting to guide diagnosis and management. Screening for ILD may also be appropriate in asymptomatic patients with high-risk features.