Muehlenbachs Atis, Bollweg Brigid C, Schulz Thadeus J, Forrester Joseph D, DeLeon Carnes Marlene, Molins Claudia, Ray Gregory S, Cummings Peter M, Ritter Jana M, Blau Dianna M, Andrew Thomas A, Prial Margaret, Ng Dianna L, Prahlow Joseph A, Sanders Jeanine H, Shieh Wun Ju, Paddock Christopher D, Schriefer Martin E, Mead Paul, Zaki Sherif R
Infectious Diseases Pathology Branch, Division of High Consequence Pathogens and Pathology, Centers for Disease Control and Prevention, Atlanta, Georgia.
Infectious Diseases Pathology Branch, Division of High Consequence Pathogens and Pathology, Centers for Disease Control and Prevention, Atlanta, Georgia.
Am J Pathol. 2016 May;186(5):1195-205. doi: 10.1016/j.ajpath.2015.12.027. Epub 2016 Mar 8.
Fatal Lyme carditis caused by the spirochete Borrelia burgdorferi rarely is identified. Here, we describe the pathologic, immunohistochemical, and molecular findings of five case patients. These sudden cardiac deaths associated with Lyme carditis occurred from late summer to fall, ages ranged from young adult to late 40s, and four patients were men. Autopsy tissue samples were evaluated by light microscopy, Warthin-Starry stain, immunohistochemistry, and PCR for B. burgdorferi, and immunohistochemistry for complement components C4d and C9, CD3, CD79a, and decorin. Post-mortem blood was tested by serology. Interstitial lymphocytic pancarditis in a relatively characteristic road map distribution was present in all cases. Cardiomyocyte necrosis was minimal, T cells outnumbered B cells, plasma cells were prominent, and mild fibrosis was present. Spirochetes in the cardiac interstitium associated with collagen fibers and co-localized with decorin. Rare spirochetes were seen in the leptomeninges of two cases by immunohistochemistry. Spirochetes were not seen in other organs examined, and joint tissue was not available for evaluation. Although rare, sudden cardiac death caused by Lyme disease might be an under-recognized entity and is characterized by pancarditis and marked tropism of spirochetes for cardiac tissues.
由伯氏疏螺旋体引起的致命性莱姆心肌炎鲜有确诊病例。在此，我们描述了5例患者的病理学、免疫组织化学及分子学检查结果。这些与莱姆心肌炎相关的心脏性猝死发生于夏末至秋季，患者年龄从青年到40多岁晚期不等，其中4例为男性。尸检组织样本通过光学显微镜、Warthin-Starry染色、免疫组织化学以及针对伯氏疏螺旋体的聚合酶链反应（PCR）进行评估，同时对补体成分C4d和C9、CD3、CD79a以及核心蛋白聚糖进行免疫组织化学检测。死后血液进行血清学检测。所有病例均存在具有相对特征性的地图状分布的间质性淋巴细胞性全心肌炎。心肌细胞坏死轻微，T细胞数量超过B细胞，浆细胞突出，且存在轻度纤维化。心脏间质中的螺旋体与胶原纤维相关，并与核心蛋白聚糖共定位。通过免疫组织化学在2例患者的软脑膜中发现罕见的螺旋体。在检查的其他器官中未发现螺旋体，且无法获取关节组织进行评估。尽管罕见，但莱姆病导致的心脏性猝死可能是一个未被充分认识的实体，其特征为全心肌炎以及螺旋体对心脏组织的显著嗜性。
