Pulmonary hypertension often complicates idiopathic interstitial pneumonia, leading to increased morbidity and mortality. Currently, there are no proven treatments for Interstitial Pneumonia-Associated Pulmonary Hypertension (PH-IIP). This study aims to evaluate the efficacy of riociguat, a pulmonary hypertension drug, in patients with PH-IIP.

This randomized, double-blind, placebo-controlled study included a total of 147 participants with PH-IIP. The patients were randomly assigned in a 1:1 ratio to receive riociguat or placebo for 26 weeks. The primary outcome of the study was a change in 6-min walking distance (6MWD). Adverse events and serious adverse events were also evaluated.

The study was terminated at mean treatment duration of 157 days because of the increased serious adverse events. During the treatment duration, 37% of the patients in the riociguat group and 23% of the patients in the placebo group experienced serious adverse events, including 11 deaths in the riociguat group. No signals of efficacy were found during the study. Commonly occurring adverse events were edema and diarrhea. Serious adverse events were the worsening of interstitial lung disease and pneumonia.

The research concluded that riociguat was associated with a high risk of severe adverse events and insignificant efficacy in patients with PH-IPP.

Ref: https://www.thelancet.com/journals/lanres/article/PIIS2213-2600(19)30250-4/fulltext