UCLA

Background: Survival in patients with systemic sclerosis (SSc) associated pulmonary hypertension (PH) and interstitial lung disease (ILD) [SSc-PH-ILD] is poor. This study investigates transplant-free survival in patients with isolated SSc pulmonary arterial hypertension (SSc-PAH) and SSc-PH-ILD treated with aggressive PAH-targeted therapy.

Methods: Kaplan-Meier and Cox proportional hazards models were constructed to analyze survival and identify predictive variables.

Results: The 1- and 2-year survival estimates were 72% and 59% versus 82% and 66% for the SSc-PH-ILD and SSc-PAH groups, respectively (p=0.5). In the Cox model, male gender (HR 0.7; p=0.01) and prostanoid therapy initiation within 6 months of the RHC (HR 1.4; p=0.01) were the only factors significantly associated with transplant-free survival, after accounting for the presence of ILD and severity of PH.

Conclusion: Survival of patients with SSc-PH-ILD has improved relative to historical series and may in part be due to aggressive PAH-targeted therapy.