Lin Xijun, Lin Peiliang, Liu Xiang
Department of Otolaryngology Head and Neck Surgery,Sun Yat-sen Memorial Hospital,Sun Yat-sen University,Guangzhou,510000,China.
Lin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2022 Apr;36(4):293-296. doi: 10.13201/j.issn.2096-7993.2022.04.011.
The purpose of this article was to discuss the clinical features and imaging characteristics of IgG4-related disease(IgG4-RD) in order to identify nasopharyngeal IgG4-RD at an early stage. The basic information of the patients, including age, sex, symptoms, disease duration and treatment process, was collected through the electronic case system. Laboratory tests including nasal endoscopy, EBV levels, IgG4 levels and C-reactive protein levels were recorded during hospitalization and outpatient follow-up. All radiological imaging and postoperative pathology data are collected, analyzed and summarized. All patients underwent partial excisional biopsy of the lesion. The pathological findings showed inflammatory granulomatous and fibrous tissue hyperplasia with a high infiltration of lymphocytes, plasma cells and neutrophils, and immunohistochemistry examination showed IgG4+ plasma cells were more than 10 per high magnification field. Combining medical history, imaging, serological findings and relevant treatment, all four patients were diagnosed with IgG4-associated disease. And their symptoms improved significantly after hormonal and immunosuppressive treatment. IgG4-RD has a highly similar clinical presentation with nasopharyngeal carcinoma. Differentiation from IgG4-RD should be considered for those pathology cannot be clarified by multiple biopsies. Timely diagnosis of IgG4-RD is important to prevent secondary organ damage in patients with active disease.
本文旨在探讨IgG4相关疾病(IgG4-RD)的临床特征及影像学特点,以便早期识别鼻咽部IgG4-RD。通过电子病历系统收集患者的基本信息,包括年龄、性别、症状、病程及治疗过程。住院期间及门诊随访时记录实验室检查结果,包括鼻内镜检查、EB病毒水平、IgG4水平及C反应蛋白水平。收集、分析并总结所有影像学检查及术后病理数据。所有患者均接受病变部位的部分切除活检。病理结果显示炎症性肉芽肿及纤维组织增生,淋巴细胞、浆细胞及中性粒细胞浸润明显,免疫组化检查显示每高倍视野IgG4+浆细胞超过10个。结合病史、影像学、血清学检查结果及相关治疗,4例患者均诊断为IgG4相关性疾病。经激素及免疫抑制治疗后,患者症状明显改善。IgG4-RD与鼻咽癌临床表现高度相似。对于多次活检仍无法明确病理的患者,应考虑与IgG4-RD相鉴别。及时诊断IgG4-RD对于预防活动性疾病患者的继发性器官损害至关重要。