Rheumatology Advance Access originally published online on January 27, 2009
Rheumatology 2009 48(3):304-308; doi:10.1093/rheumatology/ken488
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study
1Internal Medicine, Hôpital Claude Huriez, Lille, 2Vascular Medicine, Hôpital Nord, Grenoble, 3Actelion Pharmaceuticals France, Paris, 4Internal Medicine, Hôpital Bretonneau, Tours, 5Rheumatology, Hôpital Cochin, Paris, 6Rheumatology, Hôpital de Hautepierre, Strasbourg, 7Internal Medicine, Hôpital Cochin, Paris, 8Internal Medicine, Hôpital Sud, Rennes, 9Internal Medicine, Hôpital Saint Antoine, Paris, 10Cardiology, Hôpital Cardiologique, Lille, 11Internal Medicine, Pavillon des Médecines, Hôpital de Toulouse-Purpan, Toulouse, 12Service de Médecine Vasculaire, Groupe Hospitalier Saint-Joseph, Paris, 13Orgamétrie, Roubaix and 14Université Paris-Sud 11, Respiratory, Hôpital Antoine Béclère, Assistance Publique Hôpitaux de Paris, Clamart, France.
Correspondence to: Eric Hachulla, Department of Internal Medicine, National Reference Center for Scleroderma, Claude Huriez Hospital, University of Lille, 59037 Lille cedex, France. E-mail: ehachulla{at}chru-lille.fr
 |
Abstract |
|---|
Objectives. This longitudinal study investigated survival, risk factors and causes of death in the multicentre ItinérAIR-Sclérodermie cohort of patients with SSc without severe pulmonary fibrosis or severe left heart disease at baseline.
Methods. At 3-year follow-up, vital status was obtained from investigators or French national death records. Causes of death were classified as SSc-related or otherwise. Data were censored at 37 months, time of death or loss to follow-up, whichever was earlier. Survival was estimated using the Kaplan–Meier method. Multivariate survival analyses were conducted using the Cox model.
Results. In total, 546 patients were followed for a median duration of 37 months, representing 1547 patient-years. At baseline, the majority of patients were female, with lcSSc, mean age 54.9 ± 13.0 years and mean duration of SSc of 8.8 ± 8.1 years. In total, 47 patients died, giving a 3-year survival of 91.1% and cumulative mortality of 3.04 deaths per 100 patient-years; 17 deaths (32.2%) resulted from pulmonary arterial hypertension (PAH) and eight (17.1%) from cancer. Of the 47 patients with PAH at baseline, 20 died during follow-up, giving a 3-year survival of 56.3%. In a multivariate analysis, PAH [hazard ratio (HR) 7.246], age at first symptom (HR 1.052), duration of SSc (HR 1.047 per year) and Rodnan skin score (per one point) (HR 1.045) were associated with increased mortality.
Conclusion. This 3-year study observed survival and mortality estimates that were comparable with previous reports. PAH increased the HR for mortality in patients with SSc, justifying yearly echocardiographic screening.
KEY WORDS: Systemic sclerosis, Survival, Pulmonary arterial hypertension, Pulmonary hypertension
Submitted 2 August 2008;
revised version accepted 1 December 2008.
CiteULike 
Connotea 
Del.icio.us What's this?