ABSTRACT
Pirfenidone is an anti-fibrotic drug that has been shown to reduce the decline of lung function in patients with idiopathic pulmonary fibrosis (IPF) in multiple clinical trials, and has become first-line drugs for the treatment of IPF. Similar to the pathogenesis of IPF, clinical incidence of connective tissue disease-associated interstitial lung diseases (CTD-ILD) is high, and the clinical performance is not obvious. ILD may be the only or the original manifestation. Thus the clinical heterogeneity and the misdiagnosis rate are relatively high. Also, CTD-ILD is one of the important reasons that resulted in the death of patients. Currently, the clinical diagnosis and treatment of CTD-ILD still lack effective guide or unified agreement. Studies have shown that anti fibrosis drug pirfenidone shows some potential in the treatment of CTD-ILD. Here we summarize the research advances of pirfenidone in the treatment of CTD-ILD.