"The findings demonstrated that in patients with PH-IIP, riociguat was associated with increased serious adverse events and mortality, and an unfavorable risk-benefit profile. Riociguat should not be used in patients with PH-IIP," concluded the authors.

"Idiopathic interstitial pneumonia, especially idiopathic pulmonary fibrosis, are frequently accompanied by treatment-refractory, WHO group III pulmonary hypertension, and these patients are more likely to have a poor prognosis over time compared with patients whose disease course is not complicated by pulmonary hypertension. Although the antifibrotic agents pirfenidone and nintedanib substantially improve lung function decline in patients with idiopathic pulmonary fibrosis and might benefit patients with non-idiopathic forms of pulmonary fibrosis, the search for a beneficial agent that ameliorates pulmonary hypertension that can arise in patients with idiopathic interstitial pneumonia has yet to find an effective pharmacotherapy," Keith C Meyer, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA, writes in an accompanying editorial.

To read the complete study log on to https://doi.org/10.1016/S2213-2600(19)30250-4