IgG4相关性疾病累及肾盂一例报告并文献复习  

IgG4 related disease involving the renal pelvis： a case report and the literature review

作　　者：丁振山[1] 陈星[1] 冯照晗[1] 张晓云 宋黎明[2] 邢念增[2] 张冠[1] 

机构地区：[1]中日友好医院泌尿外科,北京100029 [2]首都医科大学附属北京朝阳医院泌尿外科

出　　处：《中华泌尿外科杂志》2018年第1期6-9,共4页Chinese Journal of Urology

摘　　要：目的探讨IgG4相关性疾病累及肾盂的疾病特点及诊治方法。方法回顾性分析2016年10月12日收治的1例IgG4相关性疾病累及肾盂患者的临床资料。男，56岁。因体检发现尿潜血阳性1个月就诊。泌尿系CT检查：平扫示右肾盂、输尿管壁增厚，局部软组织密度；增强扫描示肾盂、输尿管壁明显增厚，渐进性强化，考虑肾盂癌，肾门淋巴结转移。PET-CT（18F-FDG）检查：右肾门软组织肿块，右肾盂、输尿管上段管壁增厚，葡萄糖代谢增加，考虑恶性病变。术前诊断为腹膜后占位性病变、肾盂癌可能、淋巴瘤可能。患者拒绝输尿管镜检查明确诊断，遂行3D腹腔镜下肾输尿管全长切除术。术中发现右肾门及腹膜后淋巴结广泛肿大，肾门及肾盂处肿块与下腔静脉、肾动静脉粘连严重。结果术后病理检查：肾门处淋巴组织高度增生，伴生发中心形成。免疫组化染色检查示大部分浆细胞IgG4表达阳性（IgG4〉40%，IgG4〉100个/HPF），淋巴结反应性增生。考虑为IgG4相关性疾病。测定血清IgG4 15.2 g/L（1 520 mg/dl,正常值0.03～2.01 g/L）。结合病理检查和术后血清学检查结果，确诊为IgG4相关性疾病。患者术后1个月复查血清IgG4 1.95 g/L（195 mg/dl），较术前下降明显，无其他器官受累证据，未行辅助治疗。结论IgG4相关性疾病原因不明，由大量淋巴细胞和IgG4阳性浆细胞浸润，导致器官肿大或结节性/增生性病变，同时伴组织纤维化。累及肾盂可表现为肾盂软组织肿块，易被误诊为恶性肿瘤，通过详细的影像学、血清学等检查可协助确诊，避免不必要的手术。ObjectiveTo summarize the characteristics of IgG4 related disease（IgG4-RD）, and to avoid unnecessary surgical procedures due to misdiagnosis.MethodsRetrospective analysis of the clinical data of one case of IgG4 related disease involving the renal pelvis in our hospital, and the clinical features of IgG4 related disease involving the renal pelvis were reviewed and discussed. A 56-year-old man presented with microscopic hematuria, CT showed right renal pelvis and ureteral wall thickening, local soft tissue density, post contrast CT showed low-density renal pelvic mass and the wall of the ureter-pelvic was irregularly thicken, indicating renal pelvic cancer and lymph node metastasis. PET-CT（18F-FDG） findings indicated that the renal pelvic mass was a malignant tumor, because the glucose metabolism was very high. The preoperative diagnosis was retroperitoneal lesion, suspecting renal pelvic carcinoma or lymphoma. The patient underwent 3D laparoscopic nephroureterectomy with out preoperative biopsy. During operation, we found extensive enlargement of the right renal portal and retroperitoneal lymph nodes, including that the renal portal and the renal pelvis mass had a serious adhesion to the vena cava and renal arterial vein.ResultsPostoperative pathology results revealed lymphatic tissue hyperplasia and germinal center formation. Immunohistochemical staining showed that most plasma cells positive expression of IgG4 （IgG4〉40%, IgG4〉100 /HPF）, reactive hyperplasia of lymph node, considering the IgG4 related diseases. Serum IgG4 level was 15.2 g/L（0.03-2.01 g/L）after operation. Combined with serological examination, IgG4 related disease was confirmed. There was no evidence of IgG4-related disease at any other site during the follow up period, thereby obviating the need for any additional therapy.ConclusionsIgG4 related disease is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells, which leads to the

关 键 词：IGG4相关性疾病 肾盂癌 外科手术 诊疗 

分 类 号：R737.11[医药卫生—肿瘤]