PMID- 30332670 OWN - NLM STAT- MEDLINE DCOM- 20200427 LR - 20220409 IS - 1423-0356 (Electronic) IS - 0025-7931 (Linking) VI - 97 IP - 3 DP - 2019 TI - Efficacy and Safety of Pirfenidone in Advanced Idiopathic Pulmonary Fibrosis. PG - 242-251 LID - 10.1159/000492937 [doi] AB - BACKGROUND: Although phase 3 trials showed significant efficacy and acceptable safety profiles for pirfenidone in mild-to-moderate idiopathic pulmonary fibrosis (IPF), data on advanced IPF are limited. OBJECTIVES: The study aimed to evaluate the efficacy and safety of pirfenidone in advanced IPF patients. METHODS: The clinical data of 138 IPF patients (advanced group: 27%) treated with pirfenidone were retrospectively reviewed and compared between advanced and non-advanced groups. Advanced IPF was defined as (1) forced vital capacity (FVC) < 50% predicted or (2) diffusing capacity for carbon monoxide < 30% predicted. RESULTS: The mean treatment duration was 51.3 weeks, and lung function analysis was performed in 81 patients (17 in the advanced group). Changes in FVC and total lung capacity (TLC) were significantly reduced at 6 months after treatment in both the advanced (DeltaFVC [6 months]: -6.3 [before] vs. 0.7% predicted [after]; DeltaTLC: -5.3 vs. 0.8) and non-advanced (DeltaFVC: -3.4 vs. 0.5; DeltaTLC: -3.1 vs. -0.9) groups. The rate of decline in FVC and TLC was significant before treatment, but not after treatment in the advanced (FVC: -1.27 [before] vs. 0.21% predicted/month [after]; TLC: -0.89 vs. -0.15) and non-advanced (FVC: -0.60 vs. -0.20; TLC: -0.54 vs. -0.17) groups. The advanced group showed a similar rate of adverse events (AEs) (78.4 vs. 88.1%, p = 0.270), but more serious AEs (40.5 vs. 10.9%, p < 0.001) including death (24.3 vs. 5.0%, p = 0.002). CONCLUSIONS: In advanced IPF, pirfenidone showed similar efficacy and safety to non-advanced IPF except for serious AEs, which may be due to the advanced status itself. CI - (c) 2018 S. Karger AG, Basel. FAU - Yoon, Hee-Young AU - Yoon HY FAU - Kim, Dong Soon AU - Kim DS FAU - Song, Jin Woo AU - Song JW LA - eng PT - Journal Article DEP - 20181017 PL - Switzerland TA - Respiration JT - Respiration; international review of thoracic diseases JID - 0137356 RN - 0 (Anti-Inflammatory Agents, Non-Steroidal) RN - 0 (Pyridones) RN - D7NLD2JX7U (pirfenidone) SB - IM CIN - Respiration. 2019;97(3):202-204. PMID: 30453286 MH - Aged MH - Anti-Inflammatory Agents, Non-Steroidal/administration & dosage MH - Disease Progression MH - Dose-Response Relationship, Drug MH - Female MH - Follow-Up Studies MH - Humans MH - Idiopathic Pulmonary Fibrosis/diagnosis/*drug therapy/physiopathology MH - Male MH - Plethysmography MH - Pyridones/*administration & dosage MH - Retrospective Studies MH - Total Lung Capacity/*physiology MH - Treatment Outcome OTO - NOTNLM OT - Adverse effects OT - Idiopathic pulmonary fibrosis OT - Pirfenidone OT - Respiratory function test OT - Treatment outcome EDAT- 2018/10/18 06:00 MHDA- 2020/04/28 06:00 CRDT- 2018/10/18 06:00 PHST- 2018/02/07 00:00 [received] PHST- 2018/08/14 00:00 [accepted] PHST- 2018/10/18 06:00 [pubmed] PHST- 2020/04/28 06:00 [medline] PHST- 2018/10/18 06:00 [entrez] AID - 000492937 [pii] AID - 10.1159/000492937 [doi] PST - ppublish SO - Respiration. 2019;97(3):242-251. doi: 10.1159/000492937. Epub 2018 Oct 17.