心脏传导阻滞:肉芽肿性多血管炎抑或IgG4相关性疾病?  

Atrioventricular Block:Granulomatosis with Polyangiitis or IgG4⁃related diseases?

作　　者：何鑫 张桂芝 鲁涛[3] 杨德彦[4] 彭琳一 陈华 张文 曾小峰 张奉春 HE Xin;ZHANG Guizhi;LU Tao;YANG Deyan;PENG Linyi;CHEN Hua;ZHANG Wen;ZENG Xiaofeng;ZHANG Fengchun(Department of Rheumatology,Key Laboratory of Rheumatology,Ministry of Education,National Clinical Research Center for Dermatologic and Immunologic Diseases,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;State Key Laboratory of Complex Severe and Rare Diseases,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Pathology,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Cardiology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医院风湿免疫科,风湿免疫病学教育部重点实验室,国家皮肤与免疫疾病临床医学研究中心,北京100730 [2]中国医学科学院北京协和医院疑难重症及罕见病国家重点实验室,北京100730 [3]中国医学科学院北京协和医院病理科,北京100730 [4]中国医学科学院北京协和医院心内科,北京100730

出　　处：《协和医学杂志》2023年第2期379-384,共6页Medical Journal of Peking Union Medical College Hospital

基　　金：环境化学与生态毒理学国家重点实验室开放课题(KF2021-15)。

摘　　要：本文报道1例耳、眼、鼻及心脏多器官受累,血清炎症指标及IgG4升高且抗中性粒细胞胞浆抗体阴性的老年女性患者。因肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)和IgG4相关疾病(IgG4-related disease,IgG4-RD)均可导致多器官受累,二者临床表现相似且在治疗方面存在一致性,故患者疑诊为GPA合并IgG4-RD(重叠综合征),给予大剂量糖皮质激素联合环磷酰胺治疗后,临床症状得到缓解。该病例同时伴有高度房室传导阻滞(atrioventricular block,AVB)的心脏受累表现,其在GPA和IgG4-RD中均较罕见,后证实为原发疾病受累,治疗2个月后患者心律恢复正常。本文就重叠综合征的特点、该患者的诊疗过程及AVB可能的病理机制展开讨论,以期为临床诊疗提供借鉴。We reported a case of an elderly female patient with multi⁃organ involvement of the ear,eye,nose and heart,with elevated serum inflammatory markers and IgG4,and negative anti⁃neutrophil cytoplasmic antibodies.The patient was suspected to have granulomatosis with polyangiitis(GPA)combined with IgG4⁃related disease(IgG4⁃RD),because both GPA and IgG4⁃RD can lead to multi⁃organ involvement,with similar clinical manifestations and consistency in treatment.After treatment with high⁃dose glucocorticoid combined with cyclophosphamide,the clinical symptoms were relieved.The patient also had a high degree of atrioventricular block(AVB),which is rare in both GPA and IgG4⁃RD.In this paper we discuss the characteristics of the overlap syndrome,the diagnosis and treatment of this patient and the possible pathological mechanism of AVB,in order to provide reference for clinical treatment.

关 键 词：肉芽肿性多血管炎 IgG4相关疾病 心脏传导阻滞 免疫抑制治疗 

分 类 号：R593[医药卫生—内科学] R764[医药卫生—临床医学] R541