Survival among patients with systemic sclerosis-associated pulmonary arterial hypertension in the Australian scleroderma cohort study

Abstract

Background and Aims: Systemic sclerosis (scleroderma; SSc) patients can develop pulmonary arterial hypertension (PAH), which is a leading cause of death. We sought to evaluate severity, therapeutic strategy and survival of SSc-PAH in the Australian Scleroderma Cohort Study (ASCS). Methods: Among patients with 2013 American College of Rheumatology/European League Against Rheumatism-defined SSc, PAH was defined as mean pulmonary artery pressure (mPAP) ≥20 mmHg, pulmonary arterial wedge pressure (PAWP) ≤15 mmHg and pulmonary vascular resistance (PVR) >2 WU. Characteristics of those with and without PAH and those with incident PAH in 2014–2020 versus 2007–2013 were compared using descriptive sta..