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Article Dans Une Revue Rheumatology Année : 2023

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

Benjamin Chaigne
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Athenaïs Boucly
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Christian Agard
Antoine Baudet
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François Goupil
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Arnaud Maurac
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Luc Mouthon

Résumé

Objective pulmonary arterial hypertension (PAH) is a leading cause of death in mixed connective tissue disease (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, systemic lupus erythematous (SLE) patients with PAH, and systemic sclerosis (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 year, 5 years, 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 predictor of mortality in MCTD-PAH.
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hal-03973280 , version 1 (11-01-2024)

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Benjamin Chaigne, Kevin Chevalier, Athenaïs Boucly, Christian Agard, Antoine Baudet, et al.. In-depth characterization of pulmonary arterial hypertension in mixed connective tissue disease: a French national multicenter study. Rheumatology, 2023, 62 (10), pp.3261-3267. ⟨10.1093/rheumatology/kead055⟩. ⟨hal-03973280⟩
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