Clinical characteristics and survival of systemic sclerosis patients with pulmonary hypertension and elevated wedge pressure: Observations from the PHAROS cohort

摘要

Background and objectiveSystemic sclerosis (SSc) is a complex autoimmune disease commonly associated with pulmonary hypertension (PH). When associated with elevated pulmonary artery wedge pressure (PAWP), pulmonary artery pressure (PAP) is either in-proportion (post-capillary PH) or higher than expected (combined PH) relative to the increased PAWP.