Department of Clinical, Internal, Anesthesiologist and Cardiovascular Sciences, Sapienza University, Viale del Policlinico 155, 00161 Rome, Italy.
Cellular and Molecular Cardiology Lab, IRCCS "Lazzaro Spallanzani", Via Portuense, 292, 00149 Rome, Italy.
Eur Heart J. 2021 Apr 21;42(16):1609-1617. doi: 10.1093/eurheartj/ehaa973.
Necrotizing coronary vasculitis (NCV) is a rare entity usually associated to myocarditis which incidence, cause, and response to therapy is unreported.
Among 1916 patients with biopsy-proven myocarditis, 30 had NCV. Endomyocardial samples were retrospectively investigated with immunohistochemistry for toll-like receptor 4 (TLR4) and real-time polymerase chain reaction (PCR) for viral genomes. Serum samples were processed for anti-heart autoantibodies (Abs), IL-1β, IL-6, IL-8, tumour necrosis factor (TNF)-α. Identification of an immunologic pathway (including virus-negativity, TLR4-, and Ab-positivity) was followed by immunosuppression. Myocarditis-NCV cohort was followed for 6 months with 2D-echo and/or cardiac magnetic resonance and compared with 60 Myocarditis patients and 30 controls. Increase in left ventricular ejection fraction ≥10% was classified as response to therapy. Control endomyocardial biopsy followed the end of treatment. Twenty-six Myocarditis-NCV patients presented with heart failure; four with electrical instability. Cause of Myocarditis-NCV included infectious agents (10%) and immune-mediated causes (chest trauma 3%; drug hypersensitivity 7%; hypereosinophilic syndrome 3%; primary autoimmune diseases 33%, idiopathic 44%). Abs were positive in immune-mediated Myocarditis-NCV and virus-negative Myocarditis; Myocarditis-NCV patients with Ab+ presented autoreactivity in vessel walls. Toll-like receptor 4 was overexpressed in immune-mediated forms and poorly detectable in viral. Interleukin-1β was significantly higher in Myocarditis-NCV than Myocarditis, the former presenting 24% in-hospital mortality compared with 1.5% of Myocarditis cohort. Immunosuppression induced improvement of cardiac function in 88% of Myocarditis-NCV and 86% of virus-negative Myocarditis patients.
Necrotizing coronary vasculitis is histologically detectable in 1.5% of Myocarditis. Necrotizing coronary vasculitis includes viral and immune-mediated causes. Intra-hospital mortality is 24%. The immunologic pathway is associated with beneficial response to immunosuppression.
坏死性冠状动脉血管炎（NCV）是一种罕见的实体，通常与心肌炎有关，其发病率、病因和治疗反应均无报道。
在 1916 例经活检证实的心肌炎患者中，有 30 例存在 NCV。通过免疫组织化学方法检测心肌内膜样本中的 Toll 样受体 4（TLR4），并通过实时聚合酶链反应（PCR）检测病毒基因组。处理血清样本以检测抗心脏自身抗体（Abs）、白细胞介素-1β（IL-1β）、白细胞介素-6（IL-6）、白细胞介素-8（IL-8）和肿瘤坏死因子（TNF）-α。随后，对免疫途径（包括病毒阴性、TLR4 阳性和 Ab 阳性）进行免疫抑制治疗。对心肌炎-NCV 队列进行了 6 个月的二维超声心动图和/或心脏磁共振检查，并与 60 例心肌炎患者和 30 例对照组进行了比较。将左心室射血分数增加≥10%定义为治疗反应。对照组的心肌内膜活检在治疗结束后进行。26 例心肌炎-NCV 患者出现心力衰竭，4 例出现电不稳定。心肌炎-NCV 的病因包括感染性病原体（10%）和免疫介导性原因（胸部创伤 3%；药物过敏 7%；嗜酸性粒细胞增多综合征 3%；原发性自身免疫性疾病 33%，特发性 44%）。免疫介导性心肌炎-NCV 和病毒阴性心肌炎中存在 Abs 阳性，心肌炎-NCV 患者的 Ab+存在血管壁自身反应。TLR4 在免疫介导形式中过度表达，在病毒中难以检测到。与心肌炎相比，IL-1β在心肌炎-NCV 中显著升高，前者住院期间死亡率为 24%，而心肌炎组为 1.5%。免疫抑制治疗使 88%的心肌炎-NCV 患者和 86%的病毒阴性心肌炎患者的心脏功能得到改善。
坏死性冠状动脉血管炎在 1.5%的心肌炎中可通过组织学检测到。坏死性冠状动脉血管炎包括病毒和免疫介导性原因。院内死亡率为 24%。免疫途径与免疫抑制治疗的有益反应相关。
