College of Medicine, The Catholic University of Korea, Seoul, Korea.
Department of Radiology, College of Medicine, The Catholic University of Korea, Seoul, Korea.
J Int Med Res. 2024 Jun;52(6):3000605241260540. doi: 10.1177/03000605241260540.
Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by chronic activation of the immune system and a tendency to form tumorous lesions. IgG4-RD is frequently characterized by the presence of tumor-like masses affecting multiple organs and is easily mistaken for a malignant neoplasm. However, IgG4-RD affecting the appendix is extremely rare, with only seven cases reported previously. We report the case of a woman in her early 60s who presented with insidious abdominal pain and radiological findings mimicking appendiceal neoplasms. After diagnosing appendiceal neoplasms, surgery was performed. The patient had a serum IgG4 concentration of <1.35 g/L, which did not satisfy one of the three revised comprehensive diagnostic criteria for IgG4-RD. A pathological examination was conducted, and the patient was diagnosed with appendiceal IgG4-RD. To the best of our knowledge, there have been no previously reported cases of IgG4-RD affecting the appendix in patients with low serum IgG4 concentrations. This report may prove beneficial for the future understanding of IgG4-RD and for the revision of diagnostic and treatment strategies.
免疫球蛋白 G4 相关疾病（IgG4-RD）是一种纤维炎症性疾病，其特征为免疫系统的慢性激活和形成肿瘤样病变的倾向。IgG4-RD 常表现为影响多个器官的肿瘤样肿块，容易误诊为恶性肿瘤。然而，IgG4-RD 累及阑尾极为罕见，既往仅报道过 7 例。我们报告了 1 例 60 多岁女性患者，其表现为隐匿性腹痛和影像学表现类似于阑尾肿瘤。诊断为阑尾肿瘤后进行了手术。患者的血清 IgG4 浓度<1.35g/L，不符合 IgG4-RD 的三个修订综合诊断标准之一。进行了病理检查，诊断为阑尾 IgG4-RD。据我们所知，目前还没有报道过血清 IgG4 浓度低的患者发生 IgG4-RD 累及阑尾的病例。本报告可能有助于未来对 IgG4-RD 的理解，并有助于修订诊断和治疗策略。
