Lung function more stable but death risk higher in advanced IPF patients on antifibrotics

Patients with advanced idiopathic pulmonary fibrosis (IPF) at the time of initiation with nintedanib or pirfenidone demonstrate more stable lung function over time, but substantially higher risk of death, compared to those with mild-to-moderate IPF, according to the results of a recent study.

“Antifibrotic therapy with nintedanib or pirfenidone slows disease progression and reduces mortality in patients with IPF, [but] patients with advanced IPF, as defined by forced vital capacity (FVC) <50 percent and/or diffusion capacity for carbon monoxide (DLCO) <30 percent of predicted, have not been included in randomized trials,” the investigators said.

To address this, they enrolled 502 patients from IPF registries in four Nordic countries in this real-world multicentre cohort study. Linear mixed models were fitted to examine changes in FVC and DLCO over time, and Cox proportional hazards models were used to evaluate both transplant-free survival and progression- and transplant-free survival.

Sixty-six (13 percent) of the 502 patients had advanced IPF. The annual change in FVC was –125 ml (95 percent confidence interval [CI], –163 to –87) and 28 ml (95 percent CI, –96 to 152) among those with mild-to-moderate and advanced IPF, respectively.

Advanced IPF at initiation with antifibrotic therapy resulted in poorer transplant-free survival (hazard ratio [HR], 2.39, 95 percent CI, 1.66–3.43) and progression- and transplant-free survival (HR, 1.60, 95 percent CI, 1.15–2.23).

“Prospective studies are needed to determine the effect of antifibrotic therapy in patients with advanced IPF,” the investigators said.