Changes in pulmonary function test results after 1 year of therapy as predictors of survival in patients with idiopathic pulmonary fibrosis.

The study group consisted of 58 patients with idiopathic pulmonary fibrosis (IPF) recognized between 1970 and 1991 who were treated for their pulmonary disease, survived for at least 1 year from the time of initiation of treatment, and had forced vital capacity (FVC) measurements at the time of diagnosis and 9 to 15 months later. Forty-four of the patients also had a single-breath diffusing capacity (Dsb) measured initially and after 9 to 15 months of treatment and 33 patients had an arterial blood gas, breathing room air at the time of diagnosis and 9 to 15 months into therapy. Patients' conditions were classified as improved, unchanged, or worse after the year of treatment based on each of the three pulmonary function tests. A > or = 10% increase in FVC, > or = 20% increase in Dsb, and > or = 5 mm Hg decrease in alveolar-arterial difference in oxygen partial pressure [P(A-a)O2] defined improved function. A > or = 10% decrease in FVC, > or = 20% decrease in Dsb, and > or = 5 mm Hg increase in P(A-a)O2 defined worse function. Patients with < 10% change in FVC, < 20% change in Dsb, and < 5 mm Hg change in P(A-a)O2 were regarded as having unchanged conditions. Kaplan-Meier survival plots and the Cox proportional hazard regression model were used to analyze survival time in relation to change in pulmonary function after 1 year of therapy. Patients with an improved or unchanged FVC at 1 year had no difference in survival (p = 0.75), but both showed enhanced survival compared with patients with a > or = 10% reduction in FVC with 1 year of treatment (p < 0.001). Patients with an improved or unchanged Dsb at 1 year also had no difference in survival (p = 0.21) but again, both showed enhanced survival compared with patients with > or = 20% decrease in Dsb with 1 year of treatment (p < 0.001). Changes in gas exchange after 1 year of treatment did not correlate with survival in the three groups. There was a trend for longer survival in improved patients compared with those with worsening gas exchange, but the p value was not significant at 0.17. We conclude that changes in the FVC and Dsb after 1 year of treatment are strongly predictive of duration of survival in patients with IPF.