Idiopathic pulmonary fibrosis (IPF) is a type of progressive, interstitial disease isolated to the lung. Pirfenidone is a medication routinely used to treat IPF, but it’s efficacy and outcomes in advanced IPF remain uncertain. Recent studies suggest that adding sildenafil, a medicine for pulmonary hypertension, to pirfenidone may result in improved outcomes. This study aims to examine the efficacy and safety of sildenafil plus pirfenidone in patients with advanced IPF and at risk of group-3 pulmonary hypertension (PH).

This double-blind, randomized, multicenter, placebo-controlled phase-2b trial included 177 patients aged 40-80 years with advanced IPF who were at risk of group-3 PH. The patients were randomized in a 1:1 ratio to oral sildenafil tables or placebo, in addition to oral pirfenidone capsules. The primary outcome was disease progression.

No significant difference in disease progression over 52 weeks was found between the sildenafil group (73%) and the placebo group (70%). Severe treatment-related adverse events were reported in 61% of the patients in the sildenafil group, compared with 62% of patients in the placebo group.

The research concluded that adding sildenafil to pirfenidone was not associated with treatment benefits versus placebo in patients with advanced IPF.

Ref: https://www.thelancet.com/journals/lanres/article/PIIS2213-2600(20)30356-8/fulltext