Management of Connective Tissue Disease-Associated Interstitial Lung Disease

The presence of interstitial lung disease (ILD) negatively affects prognosis among patients with an underlying connective tissue disease (CTD). The initial approach to care should determine whether the CTD-ILD needs pharmacologic treatment or not. There is little direct evidence to guide who and how to treat. At present, any severe, active, and/or progressive ILD should be pharmacologically treated. Immunosuppressants and/or corticosteroids are the mainstay of pharmacologic therapy for all CTD-ILDs, whereas antifibrotics may be beneficial in some scenarios. A comprehensive and multidisciplinary approach to management is also an important aspect of patient care.