Background Pulmonary arterial hypertension (PAH) is one of the leading causes of death in systemic sclerosis (SSc). Management of patients with SSc-associated PAH (SSc-PAH) is rapidly evolving and recently reported survival is better than in historical cohorts (albeit still unsatisfactory). However, no data have been reported on purely incident cohorts of patients recruited in the modern management era (2006-2011).
Objectives This study describes the characteristics and outcome of SSc patients prospectively enrolled in the multicentre French PAH registry since 2006
Methods SSc patients (according to American College of Rheumatology and/or Leroy and Medsger’s criteria) enrolled in the registry between January 2006 and November 2009 were prospectively included if they had a precapillary PAH confirmed by right heart catheterization <1 yr prior to enrollment (incident patients with newly diagnosed PAH). Patients with interstitial lung disease (ILD) on high resolution computed tomography (HRCT) were included if forced vital capacity (FVC) >70%.
Results 100 SSc patients were included; 81% were women. Mean age at PAH diagnosis was 65±12 yrs, 86% of patients had limited cutaneous SSc, 80% were in New York Heart Association functional class (NYHA FC) III or IV and 15% had ILD on HRCT, mean FVC and the ratio of diffusing capacity of the lung for carbon monoxide/alveolar volume (DLCO/VA) were 92±20% and 54±20% of predicted values, respectively. At baseline, mean 6-minute walk distance (6MWD) was 266±119m and mean pulmonary vascular resistances were 674±346 dyn.sec.cm-5. Median follow up was 2.4 years with 45 deaths observed; overall survival was 86%, 72%, 52% and 36% at 1, 2, 3 and 4 years, respectively. Univariate analysis identified age (HR: 1.04), NYHA functional class (HR: 4,03), desaturation after 6-minute walk test (HR: 0.90), DLCO/VA (HR: 0.98), presence of an ILD (HR: 2.63) and cardiac index (HR: 0.53) as factors prognostic of survival. Other parameters did not reach statistical significance (p<0.05), including SSc subtype and mPAP.
Conclusions These results confirm the poor prognosis for incident and newly diagnosed SSc-PAH patients even in the modern era of treatment. NYHA functional class, age and presence of an ILD at the diagnosis of PAH were important prognosis factors. Poor right ventricular hemodynamic function and desaturation during the 6-min walk test were also associated with mortality. As 80% of patients are still diagnosed with a NYHA function class III/IV which appears as one of the most important prognosis factor, screening allowing an earlier diagnosis should be a priority.
Disclosure of Interest D. Launay Consultant for: GSK, Actelion, Pfizer, O. Sitbon Consultant for: Actelion, BayerSchering, GSK, Lilly, Novartis, Pfizer and United Therapeutics, J.-F. Cordier Consultant for: GSK, ACTELION, PFIZER, LILLY, E. Hachulla Consultant for: GSK, Actelion, Pfizer, L. Mouthon Consultant for: Octapharma, CSL Behring, LFB Biotechnologies, Cytheris, Actelion, Pfizer, GSK and Lilly, V. Gressin Shareholder of: actelion, Employee of: actelion, L. Rottat: None Declared, P. Clerson: None Declared, G. Simonneau Consultant for: Actelion, BayerSchering, GSK, Novartis, Pfizer and United Therapeutics, M. Humbert Consultant for: Actelion, BayerSchering, GSK, Novartis, Pfizer and United Therapeutics