累及泌尿系统的igg4相关性疾病四例及文献复习.docVIP

IgG4相关性疾病伴肾脏损害病例报告并文献复习 雒真龙1,潘昊2,裴广畅2,代维2,李月强2,汪志祥2，韩敏2,何晓峰2,吕永曼2,高红宇3* 1华中科技大学同济医学院附属同济医院消化内科 2华中科技大学同济医学院附属同济医院肾病内科 3华中科技大学同济医学院附属同济医院综合医疗科 *通讯作者:高红宇gaohongyu3613@ 国家自然科学基金项目：基金81500436 [摘要] 目的 总结IgG4相关性疾病伴肾脏损害疾病的临床特点并复习相关文献。方法 回顾性分析4例IgG4相关性疾病伴肾脏损害患者的临床表现、病理特点、实验室检查、治疗及预后情况。结果4例患者均为男性，年龄51-69岁，泌尿系统损害包括不同程度的血尿、蛋白尿、肾功能异常、梗阻性肾病。4例患者均同时存在泌尿系统外的多器官受累。所有患者均存在高球蛋白血症，血清IgG及IgG4亚型显著升高。影像学表现可分为4类：肾皮质低密度影、肾脏弥漫增大、肾盂和(或)输尿管积水、腹膜后纤维化。肾脏病理显示肾间质大量淋巴细胞、IgG4阳性的浆细胞浸润伴纤维化表现。患者对糖皮质激素治疗反应良好，临床症状及肾功能均明显改善。结论 累及泌尿系统的IgG4相关性疾病临床表现复杂多样，糖皮质激素治疗可较快缓解病情。 [关键词] IgG4相关性疾病；肾功能异常；泌尿系统；糖皮质激素 Urinary system lesions of IgG4-related disease: a retrospective analysis of 4 cases and?literature?review Luo Zhen-long1, Pan Hao2, Pei Guang-chang2, Dai Wei2, Li Yue-qiang2, WangZhi-Xiang2，Han min2, He Xiao-feng2, Lv Yong-man2, Gao Hong-yu3*. 1Department of Gastroenterology; 2 Department of Nephrology; 3 Geriatric Department Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China Corresponding author: Gao Hong-yu, Email: gaohongyu3613@ [Abstract] Objective To summarize the clinical features and review the literature of urinary system lesions in IgG4-related disease (IgG4-RD)．Methods Clinical manifestation, pathologic findings, laboratory profiles, treatment and prognosis of 4 IgG4-RD patients with urinary system involvement were analyzed．Results All four patients were males with aged 51-69 years old．Urinary system lesion included hematuria, proteinuria, renal dysfunction, obstructive nephropathy. All the patients presented multiple organ involvement simultaneously, hyperglobulinemia, elevated serum IgG and IgG4. CT image often showed hypodense shadow with renal cortex, renal swelling, hydronephrosis, and retroperitoneal fibrosis．Renal pathology revealed predominantly inflammatory cells infiltration comprising lymphocytes and IgG4-positive plasma cells with fibrosis. Patients responded well to glucocorticoids with the renal function and sy