Clin Chest Med. 2021 Jun;42(2):295-310. doi: 10.1016/j.ccm.2021.03.010.
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.