Service de Médecine Interne, Centre de Référence Maladies Autoimmunes Systémiques Rares d'Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France.
APHP-CUP, Hôpital Cochin, Université Paris Cité, Paris, France.
Rheumatology (Oxford). 2023 Oct 3;62(10):3261-3267. doi: 10.1093/rheumatology/kead055.
Pulmonary arterial hypertension (PAH) is a leading cause of death in MCTD. We aimed to describe PAH in well-characterized MCTD patients.
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.
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.
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.
肺动脉高压（PAH）是 MCTD 的主要死亡原因。我们旨在描述特征明确的 MCTD 患者中的 PAH。
法国肺动脉高压登记处中纳入了通过右心导管检查确诊为 PAH 的 MCTD 患者，并将其与匹配的对照组进行比较：无 PAH 的 MCTD 患者、PAH 的 SLE 患者和 PAH 的 SSc 患者。使用 Kaplan-Meier 方法估计生存率，并通过多变量分析寻找 MCTD 患者中 PAH 的危险因素和 MCTD-PAH 患者的死亡率的危险因素。
研究纳入了 36 例 MCTD-PAH 患者。与无 PAH 的 MCTD 患者比较和多变量分析显示，心包炎、多发性关节炎、血小板减少症、间质性肺病（ILD）和抗 Sm 抗体是 MCTD 中 PAH/PH 的独立预测因素。PAH 诊断后 1、5 和 10 年的估计生存率分别为 83%、67%和 56%。MCTD-PAH 的表现和生存率与 SLE-PAH 和 SSc-PAH 无差异。多变量分析显示，吸烟是 MCTD-PAH 死亡率的独立预测因素。
PAH 是 MCTD 的罕见且严重的并发症，10 年生存率为 56%。我们确定了 ILD、心包炎、血小板减少症和抗 Sm 抗体是 MCTD 中 PAH 的危险因素，吸烟是 MCTD-PAH 死亡率的预测因素。
