The Efficacy and Safety of Long-term Pirfenidone Therapy in Patients with Idiopathic Pulmonary Fibrosis

Abstract

Objective Pirfenidone (PFD) is often used for years, but the efficacy and safety of long-term PFD therapy in patients with idiopathic pulmonary fibrosis (IPF) are not fully understood. Methods and Patients We retrospectively evaluated 46 patients with IPF who received PFD between February 2009 and August 2014. The efficacy and safety of PFD therapy were compared between 2 groups: long-term therapy patients who received PFD for over 1 year (group L, n=30, 65%) and short-term therapy patients who could not receive PFD for more than 1 year due to worsening of their condition or side effects (group S, n=16, 35%). Results The median age of the 46 patients was 70.5 years, and the median baseline % predicted forced vital capacity (%FVC) was 70.0%. The changes in the FVC in group L were -120 mL and -170 mL at 12 and 24 months after receiving PFD, respectively. The respective median survival times after PFD therapy in groups L and S were 1,612 days and 285 days (p<0.001). The patients in group L experienced a longer time free of acute exacerbation of IPF than those in group S (947 days vs. 145 days, p=0.001). A multivariate analysis revealed that %FVC <60% was a predictor of the inability to receive PFD for over 1 year (odds ratio 0.240, 95% confidence interval 0.060-0.958; p=0.043). With regard to grade 3-5 adverse events, only one patient exhibited grade 3 hyponatremia. Conclusion Long-term PFD therapy is effective, with few severe adverse events.

Efficacy of lower dose Pirfenidone for idiopathic pulmonary fibrosis in real practice: a retrospective cohort study

“…Although there have been several real-world studies of pirfenidone use in IPF (4,10,11,16,17), those studies did not focus on the dose of pirfenidone. Our study comprehensively investigated the effect of the dose of pirfenidone on the pulmonary function of IPF patients from three tertiary referral hospitals in real-world conditions.…”

Prevention of tracheal stenosis with pirfenidone after tracheotomy: An experimental study

“…Pirfenidone is approved by the FDA to be used in the treatment of idiopathic pulmonary fibrosis. The data reported thus far by the studies conducted on pirfenidone inform that it has a minimum level of side effects, including diarrhea, nausea, photosensitivity, rash, and vomiting …”

“…The data reported thus far by the studies conducted on pirfenidone inform that it has a minimum level of side effects, including diarrhea, nausea, photosensitivity, rash, and vomiting. 24,25 After it was discovered that pirfenidone had antifibrotic effects on the pulmonary fibrosis induced by bleomycin in hamsters, it was studied in several models of fibrotic diseases, demonstrating its antifibrotic, antiinflammatory, and antioxidant effects. 10 Liu et al 26 conducted a study on lung transplantation on two different species of rats and demonstrated that 0.5% pirfenidone administered in the food inhibited peribronchial and interstitial fibrosis.…”

Antifibrotic therapy of idiopathic pulmonary fibrosis: efficiency / safety ratio

“…При проведении многофакторного анализа, оценивающего влияние возраста, статуса курения, ВРКТ-картины, DLco и др. на продолжительность приема пирфенидона, показано, что только значение ФЖЕЛ < 60% является предиктором досрочного прекращения приема препарата [25].…”

“…пациентами, получавшими плацебо. Спустя 1 год терапии в группе пирфенидона ФЖЕЛ увеличилась на 2,3±7%, а DLco снизилась на 1,3 ± 12,2% по сравнению с исходными показателями, в то время как в группе плацебо ФЖЕЛ и DLco снизились, соответственно, на 3,3 ± 6,2% (р = 0,03) и 5,3 ± 9,8% (р = 0,3) соответственно[24].В исследование Ogawa et al было отобрано 46 пациентов с ИЛФ, получающих пирфенидон, 30 из них получали препарат более года (1-я группа, средняя продолжительность приема пирфенидона 898 дней), 16 -менее года по разным причинам (2-я группа, средняя продолжительность приема препарата 159 дней)[25].…”