SURVIVAL IN SYSTEMIC SCLEROSIS RELATED PULMONARY ARTERIAL HYPERTENSION IN THE MODERN TREATMENT ERA: RESULTS FROM A MULTICENTRE AUSTRALIAN COHORT STUDY

Abstract

Background Pulmonary arterial hypertension (PAH) is the leading cause of mortality in patients with systemic sclerosis (SSc). Objectives To determine the cumulative survival rates and predictors of mortality in SSc- PAH Methods Patients enrolled in a SSc longitudinal cohort between 2007 and 2015 were included. PAH was diagnosed on right heart catheterization (RHC) (mPAP >25 and PAWP <15 mmHg). Patients with pulmonary hypertension secondary to interstitial lung disease (ILD) and co-existent PAH and severe interstitial lung disease (ILD) (defined as a HRCT scan demonstrating ILD with a FVC60%) 2.83 (1.4–5.6) 0.01 WHO functional class 2.01 (1.1–3.9) 0.03 Pulmonary arterial pressure, mmHg 1.06 (..