title: Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL registry.
abstract: BACKGROUND
  Patients with pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc-APAH) experience higher mortality rates than patients with idiopathic disease and those with other connective tissue diseases (CTD-APAH). We sought to identify unique predictors of mortality associated with SSc-APAH in the CTD-APAH population.
  
  
  METHODS
  The Registry to Evaluate Early and Long-Term PAH Management (REVEAL Registry) is a multicenter, prospective US-based registry of patients with previously and newly diagnosed (enrollment within 90 days of diagnostic right-sided heart catheterization) PAH. Cox regression models evaluated all previously identified candidate predictors of mortality in the overall REVEAL Registry population to identify significant predictors of mortality in the SSc-APAH (n = 500) vs non-SSc-CTD-APAH (n = 304) populations.
  
  
  RESULTS
  Three-year survival rates in the previously diagnosed and newly diagnosed SSc-APAH group were 61.4% ± 2.7% and 51.2% ± 4.0%, respectively, compared with 80.9% ± 2.7% and 76.4% ± 4.6%, respectively, in the non-SSc-CTD-APAH group (P < .001). In multivariate analyses, men aged > 60 years, systolic BP (SBP) ≤ 110 mm Hg, 6-min walk distance (6MWD) < 165 m, mean right atrial pressure (mRAP) > 20 mm Hg within 1 year, and pulmonary vascular resistance (PVR) > 32 Wood units remained unique predictors of mortality in the SSc-APAH group; 6MWD ≥ 440 m was protective in the non-SSc-CTD-APAH group, but not the SSc-APAH group.
  
  
  CONCLUSIONS
  Patients with SSc-APAH have higher mortality rates than patients with non-SSc-CTD-APAH. Identifying patients with SSc-APAH who are at a particularly high risk of death, including elderly men and patients with low baseline SBP or 6MWD, or markedly elevated mRAP or PVR, will enable physicians to identify patients who may benefit from closer monitoring and more aggressive treatment.
  
  
  BACKGROUND
  ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov.
authors.affiliation: Division of Immunology and Rheumatology, Veteran Affairs Palo Alto Health Care System, Palo Alto, CA. Electronic address: shauwei@stanford.edu.
authors.forename: Lorinda
authors.initials: L
authors.lastname: Chung
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Boston University, Boston, MA.
authors.forename: Harrison W
authors.initials: HW
authors.lastname: Farber
authors.affiliation: Division of Cardiovascular Medicine, Allegheny General Hospital, Pittsburgh, PA.
authors.forename: Raymond
authors.initials: R
authors.lastname: Benza
authors.affiliation: ICON Clinical Research, San Francisco, CA.
authors.forename: Dave P
authors.initials: DP
authors.lastname: Miller
authors.affiliation: ICON Clinical Research, San Francisco, CA.
authors.forename: Lori
authors.initials: L
authors.lastname: Parsons
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD.
authors.forename: Paul M
authors.initials: PM
authors.lastname: Hassoun
authors.affiliation: Division of Cardiology, Mayo Clinic, Rochester, MN.
authors.forename: Michael
authors.initials: M
authors.lastname: McGoon
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Stanford University, Stanford, CA, Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford, CA, Veteran Affairs Palo Alto Health Care System, Palo Alto, CA.
authors.forename: Mark R
authors.initials: MR
authors.lastname: Nicolls
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Stanford University, Stanford, CA, Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford, CA.
authors.forename: Roham T
authors.initials: RT
authors.lastname: Zamanian
mesh_terms: D000367:Age Factors; D000704:Analysis of Variance; D006328:Cardiac Catheterization; D002423:Cause of Death; D015331:Cohort Studies; D015897:Comorbidity; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality*; D053208:Kaplan-Meier Estimate; D008297:Male; D015999:Multivariate Analysis; D011237:Predictive Value of Tests; D016016:Proportional Hazards Models; D012042:Registries; D018570:Risk Assessment; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality*; D012720:Severity of Illness Index; D012737:Sex Factors; D016019:Survival Analysis