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

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Abstract
Objective: To examine the effect of pirfenidone on the survival of patients hospitalized due to acute exacerbation of idiopathic pulmonary fibrosis (AE–IPF). Methods: 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. Results: The treatment group had a significantly longer survival with respect to the control group [median survival time:137.0 (95%CI, 39.0 to 373.0) vs 16.0 (95%CI, 14.0 to 22.0) days; p = 0.0009]; the hazard ratio for death was 0.2896 (95% CI, 0.09541 to 0.8791). The treatment group also tended to have a lower RICU mortality rate (3/11 vs 7/9; p= 0.0698). Conclusions: Pirfenidone significantly improved survival in the IPF patients hospitalized for severe acute exacerbation with respect to their counterparts.

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