The pulmonary arterial hypertension quality enhancement research initiative: comparison of patients with idiopathic PAH to patients with systemic sclerosis-associated PAH

摘要: Objective The objective of this report is to compare baseline, management and survival characteristics in idiopathic pulmonary arterial hypertension (IPAH) with systemic sclerosis-associated (SSc-APAH) using data from the prospectively enrolled PAH Quality Enhancement Research Initiative. Methods Between August 2005 July 2007, patients IPAH SSc-APAH were across 60 US sites followed up for 3 years. Data on diagnostic tests, clinical variables, (PAH) medication outcomes recorded. Results With some exceptions, baseline laboratory similar between 279 228 SSc-APAH. Patients older at time diagnosis, more likely be female antinuclear antibody positive. had poorer spirometric results. During 3-year follow-up, both groups managed prostacyclin analogue treatment, endothelin receptor antagonists phosphodiesterase type 5 inhibitors (PDE5i) singly or combination. At years, treated PDE5i alone an antagonist. a significantly lower rate compared (60% vs 77%, p Conclusions cohort was older, severely ill, female, reduced IPAH.