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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。Abstract: 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.作者贡献：何鑫负责查阅文献，撰写论文；张桂芝、鲁涛、杨德彦、彭琳一、陈华、张文、曾小峰、张奉春负责患者诊治及病情讨论，提供论文修改建议；彭琳一、张文指导论文修订。利益冲突：所有作者均声明不存在利益冲突
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图 1 患者治疗前后心电图检查结果
A. 治疗前住院期间示Ⅲ度房室传导阻滞(2021年3月11日)；B. 治疗后1个月示Ⅱ度Ⅰ型度房室传导阻滞(2021年4月26日)；C. 治疗后5个月示Ⅰ度房室传导阻滞(2021年8月20日)
图 2 患者治疗前后胸部CT检查结果
A、B. 治疗前可见多发结节，心包少量积液(2021年2月24日)；C. 治疗后结节减少，部分结节较前缩小(2021年4月26日)；D. 治疗后心包积液消失(2021年7月13日)
图 3 患者治疗前后颞骨薄层扫描CT(冠位+轴位)检查结果
A、B. 治疗前可见双侧上颌窦炎，双侧中耳乳突炎(2021年2月24日)；C、D. 治疗后双侧上颌窦炎右侧未见，左侧明显减轻，双侧中耳乳突炎较前减轻(2021年5月26日)
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