Survival in systemic sclerosis with pulmonary arterial hypertension has not improved in the modern era.

Background: The impact of modern therapy on survival in pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc) is not clear. We sought to determine associations among commonly used clinical and hemodynamic variables, treatment, and long-term survival in PAH associated with SSc compared with PAH defined as idiopathic, familial, or associated with anorexigens.

Methods: The observation period (1996-2010) included the option for epoprostenol and the availability of oral agents in 2002 (modern era of endothelin antagonists and phosphodiesterase-5 inhibitors). Primary outcome was all-cause mortality.

Results: Eighty-three patients had SSc (mean age, 59 years), and 120 had PAH (mean age, 51 years) (P < .0001, > 80% were functional class III or IV in both groups). Compared with PAH, SSc had a lower mean pulmonary artery pressure (48 mm Hg vs 58 mm Hg, P < .0001) and pulmonary vascular resistance (10 resistance units vs 15 resistance units, P < .0001), and a higher cardiac index (2.3 L/min/m2 vs 1.8 L/min/m2, P < .0001). PAH was more often treated with prostacyclin (71% vs 44%, P < .0001), but there were no differences in the use of monotherapy or combination oral therapy. SSc had a twofold-higher mortality over the 14 years. The 5-year survival in the modern era for PAH was 87%, compared with 51% for SSc (P < .001).

Conclusions: Despite an improvement in clinical status, unlike in PAH, mortality in SSc has not improved since the introduction of epoprostenol.