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Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era
  1. David Launay1,2,
  2. Olivier Sitbon3,4,5,
  3. Eric Hachulla1,2,
  4. Luc Mouthon6,
  5. Virginie Gressin7,
  6. Laurence Rottat3,4,5,
  7. Pierre Clerson8,
  8. Jean-François Cordier9,
  9. Gerald Simonneau3,4,5,
  10. Marc Humbert3,4,5
  1. 1Service de Médecine Interne, Centre de référence de la sclérodermie systémique, Université Lille Nord de France, Hôpital Claude-Huriez, Lille, France
  2. 2Laboratoire d'Immunologie EA2686, Université Lille Nord de France, Lille, France
  3. 3Université Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre, France
  4. 4AP-HP, Service de Pneumologie, DHU Thorax Innovation, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
  5. 5INSERM U999, Centre Chirurgical Marie-Lannelongue, LabEx LERMIT, Le Plessis-Robinson, France
  6. 6Université Paris Descartes, Service de Médecine Interne, Centre de référence des vascularites nécrosantes et de la sclérodermie systémique, Université Paris Descartes, Hôpital Cochin, Paris, France
  7. 7Actelion Pharmaceuticals, Paris, France
  8. 8Orgamétrie, Roubaix, France
  9. 9Hospices Civils de Lyon, Service de Pneumologie, Centre de référence des maladies pulmonaires rares, Lyon, France
  1. Correspondence to Professor David Launay, Service de Médecine Interne, Hôpital Claude-Huriez, Rue Michel Polonovski, CHRU Lille, F-59037 Lille Cedex, France; david.launay{at}


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.

  • Systemic Sclerosis
  • Outcomes research
  • Treatment

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