Interstitial lung disease (ILD) is a well-recognised complication of connective tissue diseases (CTD), leading to significant mortality and morbidity. The pathogenesis is thought to be driven by immune-mediated inflammation and subsequent architectural damage [1]. Current pharmacotherapy options predominantly focus on the use of immune suppression, although recently antifibrotic therapy has shown some promise. This article will highlight the best available evidence for treatment options in the most common forms of CTD-ILD.