Treatment of pulmonary hypertension

Pulmonary hypertension (PH) is a significant contributor to the morbidity and mortality associated with systemic sclerosis (SSc). The spectrum of PH in SSc patients includes patients with isolated pulmonary arterial hypertension (SSc-PAH; World Health Organization Group 1) and patients with PH due to pulmonary fibrosis (SSc-ILD-PH; World Health Organization Group 3). In the last 20 years, the combined pulmonary manifestations of SSc have overtaken renal involvement as the primary cause of mortality [1, 2], accounting for up 60% of the SSc-related deaths [2]. Pulmonary arterial hypertension, independent of other pulmonary manifestations, is the second leading cause of mortality in SSc patients [2]. All forms of SSc-related PH share a grim prognosis, and the efficacy of currently available PAH-specific therapies in SSc patients has been disappointing when compared to patients with other forms of PAH.