Su W J, Chang N, He H Y
Department of Pathology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, China.
Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Department of Endoscopy, Peking University Cancer Hospital and Institute for Cancer Research, Beijing 100142, China.
Zhonghua Bing Li Xue Za Zhi. 2022 Oct 8;51(10):970-975. doi: 10.3760/cma.j.cn112151-20220325-00222.
To analyze the clinicopathological features of IgG4-related diseases (RD) of retroperitoneum and the urinary and male reproductive system (IgG4-RUMR). A total of 11 IgG4-RUMR cases from January 2013 to March 2021 were retrospectively collected at Peking University Third Hospital and Shandong Provincial Hospital affiliated to Shandong First Medical University. The clinicopathologic features, laboratory and imaging findings were analyzed and scored according to the 2019 ACR/EULAR classification criteria for IgG4-RD. The 11 patients (male:female is 9∶2; mean age 59 years, range from 44 to 83 years) were initially admitted to the Deparment of Urology/Kidney Transplantation (10 cases) and the Department of Oncology (1 case). All patients had urogenital disorders or imaging abnormalities. Three of the 11 patients had a history of IgG4-RD such as lacrimal gland engorgement, salivary gland engorgement and IgG4-associated pancreatitis. Abnormal retroperitoneal soft tissue and hydronephrosis were found in eight cases, while epididymal and spermatic cord masses were found in one case, simple renal mass in one case, and"benign prostatic hyperplasia"in one case. In the 10 patients tested for serum IgG4, the serum IgG4 level was 0.8-14.4 g/L. Histologically, all cases showed significant lymphoplasmacytic infiltration and storiform fibrosis, and some were accompanied by obliterative phlebitis. The number of IgG4 positive plasma cells was 12-155 per high-power field, and the IgG4/IgG ratio was 15%-77%. According to the 2019 ACR/EULAR IgG4-RD classification standard 11 cases scored 20-48 points, all of which met the diagnostic criteria of IgG4-RUMR. Therapeutically, the patient with a simple renal mass underwent partial nephrectomy. The patient with prostate lesion underwent transurethral resection of prostate and was initially diagnosed as nonspecific chronic prostatitis. Later, the patient was admitted again because of salivary gland swelling, and the pathologic diagnosis was amended. The patient with epididymal and spermatic cord masses participated in a clinical trial about retroperitoneal fibrosis. The remaining eight patients received symptomatic treatment such as adhesiolysis and stent placement. All the patients were subsequently treated with glucocorticoid/immunosuppressant and symptoms relieved. IgG4-RUMR is uncommon. In clinical practice, information from clinical, serologic, radiologic and pathologic evaluations must be integrated. IgG4-RUMR should be considered in the differential diagnosis of urinary and male reproductive diseases. The 2019 ACR/EULAR classification criteria for IgG4-RD, while relatively complex, are objective and practical in the diagnosis of IgG4-RUMR.
分析腹膜后及泌尿和男性生殖系统IgG4相关疾病（IgG4-RUMR）的临床病理特征。回顾性收集2013年1月至2021年3月北京大学第三医院和山东第一医科大学附属山东省立医院的11例IgG4-RUMR病例。根据2019年美国风湿病学会（ACR）/欧洲抗风湿病联盟（EULAR）IgG4相关疾病分类标准，对临床病理特征、实验室及影像学检查结果进行分析和评分。11例患者（男∶女为9∶2；平均年龄59岁，范围44～83岁）最初分别收治于泌尿外科/肾移植科（10例）和肿瘤科（1例）。所有患者均有泌尿生殖系统疾病或影像学异常。11例患者中3例有IgG4相关疾病病史，如泪腺肿大、唾液腺肿大及IgG4相关性胰腺炎。8例发现腹膜后软组织异常及肾积水，1例发现附睾及精索肿块，1例发现单纯肾肿块，1例发现“良性前列腺增生”。10例检测血清IgG4的患者，血清IgG4水平为0.8～14.4 g/L。组织学上，所有病例均表现为显著的淋巴浆细胞浸润和席纹状纤维化，部分伴有闭塞性静脉炎。每高倍视野IgG4阳性浆细胞数为12～155个，IgG4/IgG比值为15%～77%。根据2019年ACR/EULAR IgG4相关疾病分类标准，11例患者评分20～48分，均符合IgG4-RUMR诊断标准。治疗方面，单纯肾肿块患者行部分肾切除术。前列腺病变患者行经尿道前列腺切除术，最初诊断为非特异性慢性前列腺炎。后来因唾液腺肿大再次入院，病理诊断修正。附睾及精索肿块患者参加了一项关于腹膜后纤维化的临床试验。其余8例患者接受了粘连松解和支架置入等对症治疗。所有患者随后均接受糖皮质激素/免疫抑制剂治疗，症状缓解。IgG4-RUMR并不常见。在临床实践中，必须整合临床、血清学、影像学和病理学评估信息。在泌尿和男性生殖系统疾病的鉴别诊断中应考虑IgG4-RUMR。2019年ACR/EULAR IgG4相关疾病分类标准虽然相对复杂，但在IgG4-RUMR的诊断中客观实用。
