With few prospective studies available in patients with sarcoidosis-associated pulmonary hypertension (SAPH), we studied the impact of PH therapies on outcomes using real world data from 2008-2021, according to the multicentre registry (ReSAPH) protocol.

A total of 145 SAPH patients treated with pulmonary vasodilator therapy were included from 3 sarcoidosis centres. Median (range) age was 57.5 (38-80) years, 71% had WHO functional class III symptoms and baseline 6MWD was 274±145m. Average pulmonary vascular resistance (PVR) was 7.4±4.3 Wood units (WU), FVC% 62.6±19.5% and DLCO% 32.4±16.9%. UK patients (n=101) were more often male than US patients (p<0.05), Caucasian vs African American (p<0.0001) with more lung fibrosis by Scadding stage (p<0.0001) and higher PVR (p<0.0001).

Pulmonary vasodilator therapies were well tolerated. Monotherapy was used in 66% (96) of patients; most received a phosphodiesterase-5 inhibitor (PDE5i). Sequential combination therapy was used in 51 patients, with a combination of PDE5i and endothelin receptor antagonist in 94% (48).

Survival was better in patients receiving combination therapy versus monotherapy (hazard ratio for death (HR) 0.37, 95%CI: 0.13-0.99, p<0.05, univariable Cox regression). Additional univariable predictors of mortality included functional class III-IV, 6MWD<300m, lower FVC, lower DLCO and higher PVR (p<0.05). On multivariable Cox analysis, 6MWD?300m, higher DLCO, lower PVR and combination therapy remained significant predictors of survival. 

The improved survival with combination vs monotherapy calls for prospective studies in patients with SAPH.