Use of inhaled treprostinil in patients with interstitial lung disease and pulmonary hypertension: to boldly go where no other pulmonary vasodilator has gone before?

Pulmonary hypertension (PH) is a feared complication in patients with any chronic lung disease,1 but those with lung fibrosis have an especially poor mortality and the morbidity burden is very high.2 3 Up to very recently, there has been an almost nihilistic attitude towards these patients, since the lack of treatment options restricted the indication of a definitive diagnosis through right heart catheterisation (RHC) to lung transplant evaluation and enrolment in a clinical trial.4 Although with sound rationale, randomised placebo-controlled trials (RCT) with pulmonary vasodilators, have been negative or at worse detrimental5 (figure 1). That was until recently. The INCREASE study6 was the first double-blind RCT that had a positive outcome with respect to primary outcome (6-minute walking distance) as well as an improvement in brain natriuretic peptide levels and reduced clinical worsening events. Patients,...

Source: Thorax - March 15, 2024 Category: Respiratory Medicine Authors: Piccari, L., Wort, S. J. Tags: Thorax Editorial Source Type: research