title: Survival and predictors of mortality in systemic sclerosis-associated pulmonary arterial hypertension: outcomes from the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry.
abstract: OBJECTIVE
  To assess cumulative survival rates and identify independent predictors of mortality in patients with incident systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) who had undergone routine screening for PAH at SSc centers in the US.
  
  
  METHODS
  The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma registry is a prospective registry of SSc patients at high risk for PAH or with definite pulmonary hypertension diagnosed by right-sided heart catheterization within 6 months of enrollment. Only patients with World Health Organization group I PAH (mean pulmonary artery pressure >25 mm Hg and pulmonary capillary wedge pressure <15 mm Hg without significant interstitial lung disease) were included in these analyses.
  
  
  RESULTS
  In total, 131 SSc patients with incident PAH were followed for a mean ± SD of 2.0 ± 1.4 years. The 1-, 2-, and 3-year cumulative survival rates were 93%, 88%, and 75%, respectively. On multivariate analysis, age >60 years (hazard ratio [HR] 3.0, 95% confidence interval [95% CI] 1.1- 8.4), male sex (HR 3.9, 95% CI 1.1-13.9), functional class (FC) IV status (HR 6.5, 95% CI 1.8 -22.8), and diffusing capacity for carbon monoxide (DLCO) <39% predicted (HR 4.2, 95% CI 1.3-13.8) were significant predictors of mortality.
  
  
  CONCLUSIONS
  This is the largest study describing survival in patients with incident SSc-associated PAH followed up at multiple SSc centers in the US who had undergone routine screening for PAH. The survival rates were better than those reported in other recently described SSc-associated PAH cohorts. Severely reduced DLCO and FC IV status at the time of PAH diagnosis portended a poor prognosis in these patients.
authors: Lorinda
authors: L
authors: Chung
authors: Robyn T
authors: RT
authors: Domsic
authors: Bharathi
authors: B
authors: Lingala
authors: Firas
authors: F
authors: Alkassab
authors: Marcy
authors: M
authors: Bolster
authors: M E
authors: ME
authors: Csuka
authors: Chris
authors: C
authors: Derk
authors: Aryeh
authors: A
authors: Fischer
authors: Tracy
authors: T
authors: Frech
authors: Daniel E
authors: DE
authors: Furst
authors: Mardi
authors: M
authors: Gomberg-Maitland
authors: Monique
authors: M
authors: Hinchcliff
authors: Vivien
authors: V
authors: Hsu
authors: Laura K
authors: LK
authors: Hummers
authors: Dinesh
authors: D
authors: Khanna
authors: Thomas A
authors: TA
authors: Medsger
authors: Jerry A
authors: JA
authors: Molitor
authors: Ioana R
authors: IR
authors: Preston
authors: Elena
authors: E
authors: Schiopu
authors: Lee
authors: L
authors: Shapiro
authors: Richard
authors: R
authors: Silver
authors: Robert
authors: R
authors: Simms
authors: John
authors: J
authors: Varga
authors: Jessica K
authors: JK
authors: Gordon
authors: Virginia D
authors: VD
authors: Steen
mesh_terms: D000368:Aged; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology / Q000401:mortality*; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012042:Registries; D012595:Scleroderma, Systemic / Q000150:complications*; D014481:United States / Q000453:epidemiology
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