Title :

Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus Results From the French Pulmonary Hypertension Registry

Author(s) :

Hachulla, Eric [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Jais, Xavier [Auteur]
Université Paris-Sud - Paris 11 [UP11]
Cinquetti, Gael [Auteur]
Clerson, Pierre [Auteur]
Impact de l'environnement chimique sur la santé humaine - ULR 4483 [IMPECS]
Rottat, Laurence [Auteur]
Hôpital Bicêtre [AP-HP, Le Kremlin-Bicêtre]
Launay, David [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Cottin, Vincent [Auteur]
Hôpital Louis Pradel [CHU - HCL]
Habib, Gilbert [Auteur]
Hôpital de la Timone [CHU - APHM] [TIMONE]
Prevot, Gregoire [Auteur]
Service de pneumologie [Toulouse]
Chabanne, Celine [Auteur]
Service de cardiologie et maladies vasculaires [Rennes] = Cardiac, Thoracic, and Vascular Surgery [Rennes]
Fois, Elena [Auteur]
CHU Henri Mondor [Créteil]
Amoura, Zahir [Auteur]
Université Pierre et Marie Curie - Paris 6 [UPMC]
Mouthon, Luc [Auteur]
Hôpital Cochin [AP-HP]
Le Guern, Veronique [Auteur]
Hôpital Cochin [AP-HP]
Montani, David [Auteur]
Université Paris-Sud - Paris 11 [UP11]
Simonneau, Gerald [Auteur]
Université Paris-Sud - Paris 11 [UP11]
Humbert, Marc [Auteur]
Université Paris-Sud - Paris 11 [UP11]
Sobanski, Vincent [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Sitbon, Olivier [Auteur]
Université Paris-Sud - Paris 11 [UP11]

Journal title :

Chest

Abbreviated title :

Chest

Volume number :

153

Pages :

143-151

Publication date :

2018-01-01

Keyword(s) :

pulmonary arterial hypertension
survival
systemic lupus erythematosus

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Background Pulmonary arterial hypertension (PAH) is a rare complication of systemic lupus erythematosus (SLE). Methods We identified all patients with SLE and PAH (SLE-PAH) who were enrolled in the French Pulmonary ...
Show more >Background Pulmonary arterial hypertension (PAH) is a rare complication of systemic lupus erythematosus (SLE). Methods We identified all patients with SLE and PAH (SLE-PAH) who were enrolled in the French Pulmonary Hypertension Registry with a diagnosis confirmed by right heart catheterization (RHC). A control group of 101 patients with SLE without known PAH was selected from SLE expert centers participating in the Pulmonary Hypertension Registry. Survival was estimated by the Kaplan-Meier method. Hazard ratios associated with potential predictors of death were estimated using Cox proportional hazard models. Results Of the 69 patients with SLE-PAH identified in the French Pulmonary Hypertension Registry, 51 were included in the study. They did not differ from the control group regarding age, sex, or duration of SLE at the time of the analysis but had a higher frequency of anti-SSA and anti-SSB antibodies. The delay between SLE diagnosis and PAH diagnosis was 4.9 years (range, 2.8-12.9) years. The 3- and 5-year overall survival rates were 89.4% (95% CI, 76.2%-96.5%) and 83.9% (95% CI, 68.8%-92.1%), respectively. The survival rate was significantly better in patients with anti-U1-RNP antibodies (P = .04). Conclusions Patients with SLE-PAH have an overall 5-year survival rate of 83.9% after the PAH diagnosis. Anti-SSA/SSB antibodies may be a risk factor for PAH, and the presence of anti-U1-RNP antibodies appears to be a protective factor regarding survival.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Inserm
Université de Lille
CHU Lille

Collections :

• IMPact de l'Environnement Chimique sur la Santé humaine (IMPECS) - ULR 4483
• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Research team(s) :

Immunity, inflammation and fibrsis in auto and allo-reactivity

Submission date :

2019-03-01T14:34:56Z
2024-01-22T12:51:16Z