Association Between Initial Oral Therapy and Outcomes in Systemic Sclerosis-Related Pulmonary Arterial Hypertension.

ABSTRACT:

Objective

To compare time to clinical worsening (TTCW) based on initial oral therapy for pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc)-related PAH.

Methods

Using data from the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) registry (a multicenter prospective observational study enrolling SSc patients with incident pulmonary hypertension), we selected patients with group 1 PAH (World Health Organization Clinical Classification system) who received initial therapy (for 6 months) with an endothelin receptor antagonist (ERA), a phosphodiesterase 5 (PDE5) inhibitor, or a combination of these 2 agents (ERA/PDE5 inhibitor). The main outcome was TTCW, defined as the first occurrence of death, PAH-related hospit

SUBMITTER: Lammi MR 

PROVIDER: S-EPMC4996356 | biostudies-literature | 2016 Mar

REPOSITORIES: biostudies-literature