title: The pulmonary arterial hypertension quality enhancement research initiative: comparison of patients with idiopathic PAH to patients with systemic sclerosis-associated PAH.
abstract: 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.
authors: Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA. pclements@mednet.ucla.edu
authors: Philip J
authors: PJ
authors: Clements
authors: Mary
authors: M
authors: Tan
authors: Vallerie V
authors: VV
authors: McLaughlin
authors: Ronald J
authors: RJ
authors: Oudiz
authors: Victor F
authors: VF
authors: Tapson
authors: Richard N
authors: RN
authors: Channick
authors: Lewis J
authors: LJ
authors: Rubin
authors: Anatoly
authors: A
authors: Langer
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000959:Antihypertensive Agents / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D011379:Prognosis; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology; D012737:Sex Factors; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D058986:Phosphodiesterase 5 Inhibitors