He Jun-Wei, Zou Qian-Ming, Pan Jun, Wang Shu-Sheng, Xiang Song-Tao
Department of Urology, the Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510000, Guangdong Province, China.
World J Clin Cases. 2022 Mar 16;10(8):2510-2515. doi: 10.12998/wjcc.v10.i8.2510.
Immunoglobulin (Ig) G4-related disease (IgG4-RD) is an autoimmune disease associated with chronic and progressive inflammation and fibrosis. It is difficult to differentiate IgG4-RD involving the kidney from infectious diseases and malignancy on imaging.
We report the case of a 51-year-old Chinese man whose abdominal computed tomography scan showed diffuse bilateral enlargement of the kidneys and perirenal fat, thickening of the renal pelvic walls, and hydronephrosis of the right kidney. Relevant laboratory test results showed a serum creatinine level of 464 μmol/L. The patient was diagnosed with acute renal failure and was started on intermittent hemodialysis. Further tests revealed high serum IgG4 levels (20.8 g/L) and an enlarged right submaxillary lymph node. Biopsy and histopathological examination of the enlarged node led to the diagnosis of IgG4-RD. After corticosteroid therapy, his serum creatinine level quickly decreased to near normal levels.
IgG4-RD affecting the renal pelvis or perirenal fat is rare, with atypical imaging features. Multidisciplinary consultation is critical for accurate diagnosis and treatment of this disease. Suspected cases should undergo biopsy to avoid misdiagnosis.
免疫球蛋白G4相关疾病（IgG4-RD）是一种与慢性进行性炎症和纤维化相关的自身免疫性疾病。在影像学上，累及肾脏的IgG4-RD难以与感染性疾病和恶性肿瘤相鉴别。
我们报告一例51岁中国男性患者，其腹部计算机断层扫描显示双侧肾脏及肾周脂肪弥漫性增大、肾盂壁增厚以及右肾积水。相关实验室检查结果显示血清肌酐水平为464μmol/L。该患者被诊断为急性肾衰竭并开始接受间歇性血液透析。进一步检查发现血清IgG4水平升高（20.8g/L）且右颌下淋巴结肿大。对肿大淋巴结进行活检及组织病理学检查后确诊为IgG4-RD。经皮质类固醇治疗后，其血清肌酐水平迅速降至接近正常水平。
累及肾盂或肾周脂肪的IgG4-RD罕见，具有非典型影像学特征。多学科会诊对于该疾病的准确诊断和治疗至关重要。疑似病例应进行活检以避免误诊。
