Unité de Médecine Interne (UF04): CRMR MATHEC, Maladies Auto-immunes et Thérapie Cellulaire, Centre de Référence des Maladies auto-immunes systémiques Rares d'Ile-de-France, AP-HP, Hôpital St-Louis, F-75010 Paris, France; Université Paris Cité, IRSL, Recherche Clinique en hématologie, immunologie et transplantation, URP3518, F-75010 Paris, France.
Department of Cardiology, Hospital Saint-Louis, Paris, France.
Curr Res Transl Med. 2024 Sep;72(3):103449. doi: 10.1016/j.retram.2024.103449. Epub 2024 Apr 9.
Systemic sclerosis (SSc) is a rare autoimmune disease (AD), characterised by early diffuse vasculopathy, activation of the immune response and progressive skin and internal organ fibrosis. In severe progressive diffuse SSc (dSSc), autologous hematopoietic stem cell transplantation (aHSCT) improves survival, despite its own risk of complications and transplant related mortality (TRM). We present herein the case of a dSSc patient undergoing aHSCT with low dose cyclophosphamide conditioning and sudden acute myopericarditis and cardiogenic shock, four weeks after a second mRNA SARS-CoV-2 vaccine (Pfizer) injection. Four days of extracorporeal membrane oxygenation (ECMO) support during the aplasia period, allowed to observe full cardiac function recovery and progressive SSc rehabilitation with sustained disease response at 30 months follow-up. This report illustrates, for the first time to our knowledge, that ECMO can be indicated despite aplasia during aHSCT and successfully used as a bridge towards heart function recovery in highly selected and fragile AD patients. We review the factors that may contribute to endothelial and myocardial stunning and acute reversible cardiac failure in SSc and aggravate intrinsic endothelial injury during the aHSCT procedure. These classically include: cyclophosphamide drug toxicity, viral infections and autoimmune activation with disease flair per se. In the COVID-19 pandemic times, acute myocarditis due to recent viral infection or mRNA vaccine per se, must also be considered.
系统性硬化症（SSc）是一种罕见的自身免疫性疾病（AD），其特征为早期弥漫性血管病变、免疫反应激活以及进行性皮肤和内脏器官纤维化。在严重的弥漫性进行性 SSc（dSSc）中，自体造血干细胞移植（aHSCT）可改善生存，但存在自身并发症风险和移植相关死亡率（TRM）。我们在此报告一例 dSSc 患者，在接受低剂量环磷酰胺预处理和 mRNA 新冠病毒疫苗（辉瑞）第二次注射后四周，发生 aHSCT 后急性心肌炎和心源性休克。在再生障碍期，进行了四天的体外膜氧合（ECMO）支持，观察到完全的心功能恢复和进行性 SSc 康复，在 30 个月的随访中持续疾病反应。本报告首次表明，在 aHSCT 期间发生再生障碍时，可以考虑使用 ECMO，并且可以成功用于在高度选择和脆弱的 AD 患者中恢复心脏功能。我们回顾了可能导致 SSc 中血管内皮和心肌顿抑以及急性可逆性心力衰竭的因素，并在 aHSCT 过程中加重固有内皮损伤。这些因素通常包括：环磷酰胺药物毒性、病毒感染和自身免疫激活，以及疾病本身的恶化。在 COVID-19 大流行时期，还必须考虑最近病毒感染或 mRNA 疫苗本身引起的急性心肌炎。
