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

Published

Journal Article

OBJECTIVE: The objective of this report is to compare baseline, management and survival characteristics in idiopathic pulmonary arterial hypertension (IPAH) with systemic sclerosis-associated pulmonary arterial hypertension (SSc-APAH) using data from the prospectively enrolled PAH Quality Enhancement Research Initiative. METHODS: Between August 2005 and July 2007, patients with IPAH and SSc-APAH were enrolled across 60 US sites and followed up for 3 years. Data on diagnostic tests, clinical variables, pulmonary arterial hypertension (PAH) medication and outcomes were recorded. RESULTS: With some exceptions, baseline clinical and laboratory characteristics were similar between the 279 patients with IPAH and the 228 with SSc-APAH. Patients with SSc-APAH were older at the time of PAH diagnosis, were more likely to be female and were antinuclear antibody positive. Patients with SSc-APAH had poorer spirometric results. During the 3-year follow-up, both groups were managed with prostacyclin and prostacyclin analogue treatment, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors (PDE5i) singly or in combination. At 3 years, patients with SSc-APAH were more likely to be treated with PDE5i alone or with an endothelin receptor antagonist. Patients with SSc-APAH had a significantly lower survival rate compared to patients with IPAH (60% vs 77%, p<0.0001). CONCLUSIONS: The cohort with SSc-APAH was older, was more severely ill, was more likely to be female, was managed with PDE5i and had reduced 3-year survival compared with the cohort with IPAH.

Full Text

Cited Authors

  • Clements, PJ; Tan, M; McLaughlin, VV; Oudiz, RJ; Tapson, VF; Channick, RN; Rubin, LJ; Langer, A; Pulmonary Arterial Hypertension Quality Enhancement Research Initiative (PAH-QuERI) Investigators,

Published Date

  • February 2012

Published In

Volume / Issue

  • 71 / 2

Start / End Page

  • 249 - 252

PubMed ID

  • 21998119

Pubmed Central ID

  • 21998119

Electronic International Standard Serial Number (EISSN)

  • 1468-2060

International Standard Serial Number (ISSN)

  • 0003-4967

Digital Object Identifier (DOI)

  • 10.1136/annrheumdis-2011-200265

Language

  • eng