Abstract

Interstitial lung disease (ILD), which is characterized by pulmonary fibrosis, is a diverse group of disorders. Nintedanib, an antifibrotic drug, is known to attenuate disease progression in ILD with progressive fibrosis, but its efficacy in autoimmune-disease-related ILD remains uncertain. We conducted a comprehensive search for relevant randomized controlled trials, systematic reviews and meta-analyses included in PubMed, ScienceDirect and Scopus databases as of 23 June 2022 and manually reviewed reference lists. Among the 689 titles and abstracts screened, 24 studies were considered, with 4 randomized controlled trials included in our review. Nintedanib, administered at 150 mg twice daily for 52 weeks, consistently slowed forced vital capacity decline. Enhanced efficacy was observed when combining nintedanib with immunomodulators, and the most common adverse effect was diarrhea. In conclusion, our study suggests that nintedanib is a safe option for mitigating the progression of autoimmune-disease-related ILD, providing valuable insights into its potential therapeutic role in this context.

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