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- Title
In-depth characterization of pulmonary arterial hypertension in mixed connective tissue disease: a French national multicentre study.
- Authors
Chaigne, Benjamin; Chevalier, Kevin; Boucly, Athenaïs; Agard, Christian; Baudet, Antoine; Bourdin, Arnaud; Chabanne, Céline; Cottin, Vincent; Fesler, Pierre; Goupil, François; Jego, Patrick; Launay, David; Lévesque, Hervé; Maurac, Arnaud; Mohamed, Shirine; Tromeur, Cécile; Rottat, Laurence; Sitbon, Olivier; Humbert, Marc; Mouthon, Luc
- Abstract
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.
- Subjects
FRANCE; MORTALITY risk factors; PULMONARY arterial hypertension; RESEARCH; PERICARDITIS; MULTIVARIATE analysis; INTERSTITIAL lung diseases; CONNECTIVE tissue diseases; RISK assessment; KAPLAN-Meier estimator; DESCRIPTIVE statistics; ARTHRITIS; THROMBOCYTOPENIA; SMOKING; SYSTEMIC lupus erythematosus; DISEASE risk factors; DISEASE complications
- Publication
Rheumatology, 2023, Vol 62, Issue 10, p3261
- ISSN
1462-0324
- Publication type
Article
- DOI
10.1093/rheumatology/kead055