• Chest · Jan 2018

    Multicenter Study Observational Study

    Pulmonary arterial hypertension associated with systemic lupus: results from the French Pulmonary Hypertension Registry.

    • Eric Hachulla, Xavier Jais, Gaël Cinquetti, Pierre Clerson, Laurence Rottat, David Launay, Vincent Cottin, Gilbert Habib, Grégoire Prevot, Céline Chabanne, Eléna Foïs, Zahir Amoura, Luc Mouthon, Véronique Le Guern, David Montani, Gérald Simonneau, Marc Humbert, Vincent Sobanski, Olivier Sitbon, and French Collaborators Recruiting Members(∗).
    • Centre de reference des Maladies Auto-immunes Systémiques Rares du Nord et Nord-Ouest (CeRAINO), Service de Médecine Interne, Hôpital Huriez, Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET), INSERM U995 - LIRIC - Lille Inflammation Research International Centre, Université de Lille, Lille, France. Electronic address: eric.hachulla@chru-lille.fr.
    • Chest. 2018 Jan 1; 153 (1): 143-151.

    BackgroundPulmonary arterial hypertension (PAH) is a rare complication of systemic lupus erythematosus (SLE).MethodsWe 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.ResultsOf 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).ConclusionsPatients 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.Copyright © 2017 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

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