Patients with severe idiopathic pulmonary fibrosis (IPF) often develop additional pulmonary hypertension (WHO classification PH group 3) or, at least, are at an increased risk for it. An international, multicenter, double-blind, randomized, and placebo-controlled clinical trial investigated whether treatment with Sildenafil (approved for PH) when administered in addition to the pulmonary fibrosis medication pirfenidone is effective. The findings have recently been published in the medical journal Lancet Respiratory Medicine (Behr et al., 2021).
In a total of 56 participating centers, 177 patients between 40 and 80 years of age were included. Patients were given either Sildenafil plus pirfenidone or placebo plus pirfenidone for a period of 52 weeks.
The addition of Sildenafil did not result in any treatment benefit compared to treatment with pirfenidone only. Nevertheless, the authors recommend conducting further investigations to determine whether such treatment might be advantageous for different subgroups of IPF. Further information on the trial can be found in the entry of the clinical trial in the register ClinicalTrials.gov under trial number NCT02951429 (Link to trial).