Riociguat delays worsening of sarcoidosis-associated pulmonary hypertension

Treatment with riociguat helps prevent clinical worsening and improve exercise capacity in patients with sarcoidosis-associated pulmonary hypertension (SAPH), according to the results of a trial.

A total of 16 patients with SAPH confirmed by right heart catheterization were randomized to receive riociguat (n=8) or placebo (n=8). All of them underwent six-minute walk distance (6MWD) test and spirometry every 8 weeks.

The primary endpoint was time to clinical worsening (TCW), which was defined by the time to the occurrence of the following: all-cause mortality, need for hospitalization due to worsening cardiopulmonary status attributable to progression of disease, >50-metre decrease in the 6MWD test, or worsening of the World Health Organization (WHO) functional class.

The two treatment groups were similar in terms of pulmonary artery mean (PA mean), pulmonary vascular resistance (PVR), initial 6MWD, and forced vital capacity (FVC) at baseline.

Clinical worsening occurred more frequently with placebo than with active treatment (five vs zero patient). Log-rank analysis revealed that riociguat patients were in the study for a significantly longer period (p=0.0124).

Meanwhile, the 6MWD jumped in the riociguat group but decreased in the placebo group (median, 42.7 m, –7.5 to 91.4 vs –55.9 m, –176.8 to 60; p=0.0149), with a difference of 94 metres (p<0.01). Furthermore, more patients in the active treatment group achieved a >30-m improvement in 6MWD (four vs one patients; p>0.05).

None of the patients treated with riociguat developed significant adverse events.