腹膜后及泌尿男性生殖系统IgG4相关性疾病11例临床病理分析

IgG4-related diseases of retroperitoneum in urinary and male reproductive system:a clinicopathological analysis of eleven cases

目的通过分析以泌尿男性生殖系统症状入院的IgG4相关性疾病(IgG4-RD)患者的临床病理特征,提高对腹膜后及泌尿男性生殖系统IgG4相关性疾病(IgG4-RUMR)的认识。方法收集北京大学第三医院和山东第一医科大学附属省立医院2013年1月至2021年3月11例IgG4-RUMR病例,分析其临床病理特征、实验室检查、影像学检查等资料,按照2019美国风湿病学会/欧洲抗风湿病联盟(ACR/EULAR)IgG4-RD分类标准评分,达到20分即诊断为IgG4-RUMR。结果11例IgG4-RUMR患者男女比为9∶2,发病年龄44~83岁,中位年龄59岁,均有明显的泌尿男性生殖系统症状或影像学异常。所有患者入院时均就诊于泌尿外科/肾移植科(10例)及肿瘤科(1例)。11例患者中3例有泪腺肿物、涎腺肿大、IgG4相关性胰腺炎等相关病史。影像学上,8例显示腹膜后软组织密度影并肾脏、输尿管积水(其中1例伴肾占位),1例显示睾丸、附睾及精索肿物,1例显示单纯肾实质占位,1例考虑前列腺增生。在接受过血清IgG4检测的10例患者中,血清IgG4水平为0.8~14.4 g/L。组织学上,11例均见显著的浆细胞浸润及席纹状纤维化,少数伴闭塞性静脉炎,IgG4阳性浆细胞数目为12~155个/HPF,IgG4/IgG比值15%~77%。根据2019 ACR/EULAR IgG4-RD分类标准评分,11例得分20~48分,均满足IgG4-RUMR的诊断标准。治疗方面:1例行肾部分切除,病理学检查提示IgG4相关性疾病后,行糖皮质激素治疗;1例行前列腺经尿道电切,病理学诊断为前列腺组织非特异性慢性炎,之后患者因涎腺肿大再次入院,复核原病理切片修正诊断为前列腺IgG4相关性疾病,行糖皮质激素治疗;1例进入腹膜后纤维化药物研究性治疗;其余8例根据病情需要行粘连松解术或输尿管支架置入解除尿路梗阻后,行糖皮质激素伴或不伴免疫抑制剂治疗;所有患者的症状均在治疗后好转。结论IgG4-RUMR发病率低,临床、影像学表现非特异,其诊断需借助临床、影像学、实验室检查及病理学综合评估。在泌尿及男性生殖系统病变中,应将IgG4-RUMR纳入鉴别诊断。另外,在IgG4相关性疾病的诊断中,2019ACR/EULAR IgG4-RD分类标准虽较复杂,但客观实用,适用于临床实践。

Objective To analyze the clinicopathological features of IgG4-related diseases(RD)of retroperitoneum and the urinary and male reproductive system(IgG4-RUMR).Methods 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.Results 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.Conclusions 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.