Treatment of pulmonary hypertension in patients with connective tissue disease and interstitial lung disease.

Background: Pulmonary hypertension (PH) in patients with connective tissue disease (CTD) can occur in isolation or concomitantly with interstitial lung disease (ILD). Targeted therapies for PH can mitigate clinical deterioration in CTD patients with isolated PH; however, the effect of these therapies in CTD patients with PH and ILD (CTD-PH-ILD) are poorly characterized.

Objective: To investigate outcomes following long-term treatment of PH in patients with CTD-PH-ILD.