Pirfenidone improves the survival of patients with idiopathic pulmonary fibrosis hospitalized for acute exacerbation.

To examine the effect of pirfenidone on the survival of patients hospitalized due to acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF). The outcomes of 11 consecutive AE-IPF patients who were receiving pirfenidone treatment when they were admitted to a respiratory intensive care unit (RICU) for acute respiratory failure (ARF) (treatment group) were retrospectively compared with those of 9 patients who were not on pirfenidone treatment at admission (control group). The study's primary outcome measure was survival following RICU admission; the patients' mortality rate and the length of time spent in the RICU were also assessed. The treatment group had significantly longer survival than the control group (median survival time: 137.0 [95% CI, 39.0-373.0] versus 16.0 [95% CI, 14.0-22.0] days;  = .0009); the hazard ratio for death was 0.2896 (95% CI, 0.09541-0.8791). The treatment group also tended to have a lower RICU mortality rate (3/11 vs. 7/9;  = .0698). Pirfenidone significantly improved survival in IPF patients hospitalized for severe acute exacerbation compared to controls.