Eric Hachulla1, Xavier Jais2, Gaël Cinquetti3, Pierre Clerson4, Laurence Rottat2, David Launay5, Vincent Cottin6, Gilbert Habib7, Grégoire Prevot8, Céline Chabanne9, Eléna Foïs10, Zahir Amoura11, Luc Mouthon12, Véronique Le Guern12, David Montani2, Gérald Simonneau2, Marc Humbert2, Vincent Sobanski5, Olivier Sitbon2. 1. 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. 2. Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre; AP-HP, Centre de reference de l'Hypertension Pulmonaire, Service de Pneumologie, DHU Thorax Innovation, Hôpital Bicêtre, Le Kremlin-Bicêtre; INSERM UMR_S999, LabEx LERMIT, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson, France. 3. Service des Maladies Infectieuses et Systémiques, Hôpital d'instruction des armées Legouest, Metz, France. 4. Soladis Clinical Studies, Roubaix, France. 5. 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. 6. Service de Pneumologie - Centre des Maladies Orphelines Pulmonaires, CHU de Lyon HCL-GH Est-Hôpital Louis Pradel, Bron, France. 7. Service de Cardiologie, CHU de Marseille - Hôpital de la Timone, Marseille, France. 8. Service de Pneumologie, Pôle voies respiratoires, Hôpital Larrey, Toulouse, France. 9. Département de Cardiologie et Maladies vasculaires, CHU de Rennes - Hôpital Pontchaillou, Rennese, France. 10. Unité des maladies génétiques du globule rouge, Hôpital Henri Mondor, Créteile, France. 11. Service de Médecine Interne 2, Centre de Référence du Lupus, Syndrome des Antiphospholipides et autres Maladies Auto-Immunes Systémiques Rares, Institut E3M, Hôpital Pitié-Salpêtrière, Paris, et Université Paris VI Pierre et Marie Curie CIMI -UPMC UMRS CR7-INSERM U1135 - CNRS, France. 12. Service de Médecine Interne, hôpital Cochin, Centre de Référence des Maladies Auto-immunes Systémiques Rares Ile de France, Université Paris Descartes, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
Abstract
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.
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.