title: Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era.
abstract: OBJECTIVE
  To assess the survival and prognostic factors in patients with newly diagnosed incident systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) in the modern management era.
  
  
  METHODS
  Prospectively enrolled SSc patients in the French PAH Network between January 2006 and November 2009, with newly diagnosed PAH and no interstitial lung disease, were analysed (85 patients, mean age 64.9±12.2 years). Median follow-up after PAH diagnosis was 2.32 years.
  
  
  RESULTS
  A majority of patients were in NYHA functional class III-IV (79%). Overall survival was 90% (95% CI 81% to 95%), 78% (95% CI 67% to 86%) and 56% (95% CI 42% to 68%) at 1, 2 and 3 years from PAH diagnosis, respectively. Age (HR: 1.05, 95% CI 1.01 to 1.09, p=0.012) and cardiac index (HR: 0.49, 95% CI 0.27 to 0.89, p=0.019) were significant predictors in the univariate analysis. We also observed strong trends for gender, SSc subtypes, New York Heart Association functional class, pulmonary vascular resistance and capacitance to be significant predictors in the univariate analysis. Conversely, six-min walk distance, mean pulmonary arterial and right atrial pressures were not significant predictors. In the multivariate model, gender was the only independent factor associated with survival (HR: 4.76, 95% CI 1.35 to 16.66, p=0.015 for male gender).
  
  
  CONCLUSIONS
  Incident SSc-associated PAH remains a devastating disease even in the modern management era. Age, male gender and cardiac index were the main prognosis factors in this cohort of patients. Early detection of less severe patients should be a priority.
authors.affiliation: Service de Médecine Interne, Centre de référence de la sclérodermie systémique, Université Lille Nord de France, Hôpital Claude-Huriez, , Lille, France.
authors.forename: David
authors.initials: D
authors.lastname: Launay
authors.forename: Olivier
authors.initials: O
authors.lastname: Sitbon
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.forename: Virginie
authors.initials: V
authors.lastname: Gressin
authors.forename: Laurence
authors.initials: L
authors.lastname: Rottat
authors.forename: Pierre
authors.initials: P
authors.lastname: Clerson
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Cordier
authors.forename: Gerald
authors.initials: G
authors.lastname: Simonneau
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
mesh_terms: D000368:Aged; D000959:Antihypertensive Agents / Q000627:therapeutic use; D002302:Cardiac Output; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis / Q000401:mortality; D012737:Sex Factors; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents