METHODS: This retrospective, observational study enrolled IPF patients seen at the time of diagnosis at a single center from 2008 to 2018. Longitudinal clinical and laboratory data were prospectively collected. We compared the clinical characteristics, survival, and pulmonary function decline between patients treated and untreated with various dose of
pirfenidone.
RESULTS: Of 295 IPF patients, 100 (33.9%) received
pirfenidone and 195 (66.1%) received no antifibrotic agent. Of the 100 patients who received
pirfenidone, 24 (24%), 50 (50%), and 26 (26%), respectively, were given 600, 1200, and 1800 mg
pirfenidone daily. The mean survival time was 57.03 ± 3.90 months in the no-antifibrotic
drug group and 73.26 ± 7.87 months in the
pirfenidone-treated group (p = 0.027). In the unadjusted analysis, the survival of the patients given
pirfenidone was significantly better (hazard ratio [HR] = 0.69, 95% confidence interval [CI]: 0.48-0.99, p = 0.04). After adjusting for age, gender, body mass index, and the GAP score [based on gender (G), age (A), and two physiological lung parameters (P)], survival remained better in the patients given
pirfenidone (HR = 0.56, 95% CI: 0.37-0.85, p = 0.006). In terms of pulmonary function, the decreases in forced vital capacity (%), forced expiratory volume in 1 s (%) and the diffusing capacity of lung for
carbon monoxide (%) were significantly smaller (p = 0.000, p = 0.001, and p = 0.007, respectively) in patients given
pirfenidone.
CONCLUSIONS: