IgG4相关疾病（IgG4-RD）的特点是器官中单个或多个肿块，可能类似于各种炎症和恶性疾病。在此，我们总结了4例具有类似鼻咽癌的侵袭性IgG4-RD表现的患者，为IgG4-RD的诊断提供一些新的视角。我们的系列中纳入了4例患者。年龄53～64岁，病程4～6个月。主要症状包括头痛、鼻漏或复视。免疫组织化学显示所有患者的 IgG4 浆细胞/HPF 均超过 10 个，血浆 IgG4 水平范围为 218 mg/dL 至 765 mg/dL。均符合IgG4-RD的诊断标准。该病例与鼻咽癌的临床表现高度相似。尽管病理学是金标准，但仍然存在局限性。血清学IgG4可以帮助明确诊断。及时诊断 IgG4-RD 对于预防活动性疾病患者继发性器官损害具有重要意义。版权所有 © 2024 Lin、Lin、Fan、Zhang、Liang、Han、Liu、Huang。

IgG4-related disease (IgG4-RD) was characterized by single or multiple masses in organs, which may mimic various inflammatory and malignant diseases. Here, we summarize 4 patients with aggressive manifestations of IgG4-RD that mimic nasopharynx cancer to provide some new sights for the diagnosis of IgG4-RD.Four patients were included in our series. The age ranged from 53 to 64 years old, and the duration of the disease ranged from 4 to 6 months. The chief complaints included headache, rhinorrhea, or diplopia. All patients had more than 10 IgG4+ plasma cells/HPF in immunohistochemistry with plasma lgG4 levels ranging from 218 mg/dL to 765 mg/dL. All of them met the diagnostic criteria of lgG4-RD.The described case is highly similar to the clinical manifestations of nasopharyngeal carcinoma. Although pathology is the gold standard, there are still limitations. Serological IgG4 can help confirm the diagnosis. Timely diagnosis of IgG4-RD is of great significance in preventing secondary organ damage in patients with active diseases.Copyright © 2024 Lin, Lin, Fan, Zhang, Liang, Han, Liu and Huang.