The efficacy and safety of pirfenidone in the treatment of HPS-related pulmonary fibrosis and Idiopathic pulmonary fibrosis: a systematic review and meta-analysis

OBJECTIVE: The incidence of idiopathic pulmonary fibrosis is increasing year by year in the world, which has a greater im-pact on the quality of life of patients. In the past, symptomatic treatment was used in clin-ical practice, but the overall effect is still not good. Multiple clinical studies have demonstrat-ed the efficacy of pirfenidone in the treatment of idiopathic pulmonary fibrosis; however, adverse reactions have been reported. We, therefore, systematically evaluated the effectiveness and safety of pirfenidone in patients with idiopathic pulmonary fibrosis.PATIENTS AND METHODS: Relevant studies were retrieved from the Embase, PubMed, Web of Science, Cochrane Library, China National Knowledge Infrastructure (CNKI), Chinese Bio-medical Literature (CBM), Wanfang and Weipu databases between January 1999 and May 2020, including the keywords "pirfenidone" and "id-iopathic pulmonary fibrosis", were included in our systematic review. Review Manager 5.4 soft-ware was used for data synthesis, and analyses of publication bias and sensitivity.RESULTS: Our systematic review included 13 studies involving a total of 13,247 patients with idiopathic pulmonary fibrosis. Pirfenidone was associated with reduced declines in vital ca-pacity (VC) and forced vital capacity (FVC) from baseline in patients with hermansky-pudlak syn-drome (HPS)-related pulmonary fibrosis and to moderate idiopathic pulmonary fibrosis (IPF). Pirfenidone treatment was associated with low-er reductions in FVC, lower reductions in 6 -min-ute walking test distance, lower decreases in minimum oxygen saturation during the 6 -min-ute walking test, lower all-cause death, lower relative risk of IPF-related death and increased progression-free survival compared to placebo. Progression-free survival was significantly lon-ger in the pirfenidone group. The incidence of gastrointestinal, skin, nervous system, and liver function-related adverse events was significant-ly higher in the pirfenidone group compared to the control group.CONCLUSIONS: Pirfenidone has efficacy in de-laying the progression of idiopathic pulmonary fi-brosis. Pirfenidone is well-tolerated by the majority of patients; however, mild adverse reactions related to the gastrointestinal tract, skin, nervous system, and liver function are common. Overall, Pirfenidone may be an effective and well-tolerated treatment option for idiopathic pulmonary fibrosis.