Title :

In-depth characterization of pulmonary arterial hypertension in mixed connective tissue disease: a French national multicenter study.

Author(s) :

Chaigne, Benjamin [Auteur]
Centre de référence des maladies auto-immunes systémiques rares d'Île-de-France / National Reference Center for Rare Systemic Autoimmune Diseases
Chevalier, Kevin [Auteur]
Centre de référence des maladies auto-immunes systémiques rares d'Île-de-France / National Reference Center for Rare Systemic Autoimmune Diseases
Boucly, Athenaïs [Auteur]
Hôpital Bicêtre [AP-HP, Le Kremlin-Bicêtre]
Agard, Christian [Auteur]
Nantes Université [Nantes Univ]
Baudet, Antoine [Auteur]
Centre régional de compétence des Maladies systémiques et auto-immunes rares de l'adulte et Maladies vasculaires rares
Bourdin, Arnaud [Auteur]
Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] [PhyMedExp]
Chabanne, Céline [Auteur]
Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Pontchaillou]
Cottin, Vincent [Auteur]
Infections Virales et Pathologie Comparée - UMR 754 [IVPC]
Fesler, Pierre [Auteur]
Hôpital Lapeyronie [CHU Montpellier]
Goupil, François [Auteur]
Centre Hospitalier Le Mans (CH Le Mans)
Jego, Patrick [Auteur]
Université de Rennes [UR]
Launay, David [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Lévesque, Hervé [Auteur]
Physiopathologie et biothérapies des maladies inflammatoires et autoimmunes
Maurac, Arnaud [Auteur]
Université de Lorraine [UL]
Mohamed, Shirine [Auteur]
Centre de référence des maladies héréditaires du métabolisme [MaMEA Nancy-Brabois]
Tromeur, Cécile [Auteur]
Groupe d'Etude de la Thrombose de Bretagne Occidentale [GETBO]
Rottat, Laurence [Auteur]
Hôpital Bicêtre [AP-HP, Le Kremlin-Bicêtre]
Sitbon, Olivier [Auteur]
Hôpital Bicêtre [AP-HP, Le Kremlin-Bicêtre]
Humbert, Marc [Auteur]
Hôpital Bicêtre [AP-HP, Le Kremlin-Bicêtre]
Mouthon, Luc [Auteur]
Centre de référence des maladies auto-immunes systémiques rares d'Île-de-France / National Reference Center for Rare Systemic Autoimmune Diseases

Journal title :

Rheumatology

Abbreviated title :

Rheumatology (Oxford)

Volume number :

62

Pages :

3261–3267

Publication date :

2023-02-28

ISSN :

1462-0332

English keyword(s) :

pulmonary arterial hypertension
MCTD
SLE
SSc
survival

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Objective Pulmonary arterial hypertension (PAH) is a leading cause of death in MCTD. We aimed to describe PAH in well-characterized MCTD patients. Methods MCTD patients enrolled in the French Pulmonary Hypertension ...
Show more >Objective Pulmonary arterial hypertension (PAH) is a leading cause of death in MCTD. We aimed to describe PAH in well-characterized MCTD patients. Methods MCTD patients enrolled in the French Pulmonary Hypertension Registry with a PAH diagnosis confirmed by right heart catheterization were included in the study and compared with matched controls: MCTD patients without PAH, SLE patients with PAH and SSc patients with PAH. Survival rates were estimated by the Kaplan–Meier method and risk factors for PAH in MCTD patients and risk factors for mortality in MCTD-PAH were sought using multivariate analyses. Results Thirty-six patients with MCTD-PAH were included in the study. Comparison with MCTD patients without PAH and multivariate analysis revealed that pericarditis, polyarthritis, thrombocytopenia, interstitial lung disease (ILD) and anti-Sm antibodies were independent predictive factors of PAH/PH in MCTD. Estimated survival rates at 1, 5 and 10 years following PAH diagnosis were 83%, 67% and 56%, respectively. MCTD-PAH presentation and survival did not differ from SLE-PAH and SSc-PAH. Multivariate analysis revealed that tobacco exposure was an independent factor predictive of mortality in MCTD-PAH. Conclusion PAH is a rare and severe complication of MCTD associated with a 56% 10-year survival. We identified ILD, pericarditis, thrombocytopenia and anti-Sm antibodies as risk factors for PAH in MCTD and tobacco exposure as a predictor of mortality in MCTD-PAH.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2024-01-11T23:25:56Z
2024-02-06T08:53:16Z