1例IgG_4相关硬化性疾病临床病理分析及文献复习  

IgG_4-related sclerosing disease:A case report and review of literature

作　　者：荣娜[1] 魏日胞[1] 王远大[1] 

机构地区：[1]解放军总医院肾病科肾脏病国家重点学科暨国家重点实验室,北京100853

出　　处：《军医进修学院学报》2011年第12期1277-1279,共3页Academic Journal of Pla Postgraduate Medical School

基　　金：国家科技重大专项(2010ZX9102-204);国家自然科学基金项目(81072914);军队中医药专项课题(10ZYZ255)~~

摘　　要：目的报告1例罕见的IgG4相关硬化性疾病多器官受累病例,提高该病的认识水平。方法分析我院2010年11月诊治并经病理学确诊的IgG4相关硬化性疾病1例,并结合国内外文献,对本病的临床表现、诊断、治疗及预后进行分析讨论。结果患者临床表现为糖尿病、肾功能受损,颈部、腹股沟等全身淋巴结肿大;血清IgG、IgG4异常增高,CT检查胰腺肿大、间质性肺炎,淋巴结活检可见IgG4阳性浆细胞浸润,肾脏活检病理为局灶硬化性肾小球肾炎;激素治疗3周肾功能正常,胰腺及淋巴结形态明显缩小,20周血IgG、IgE正常,糖尿病控制良好。结论 IgG4相关硬化性疾病是一种非常罕见的全身系统性疾病,及时应用糖皮质激素可控制病情。Objective To report a rare case of IgG4-related sclerosing disease involving multiple organs in order to improve our understanding of it.Methods A rare case of IgG4-related sclerosing disease admitted to our hospital in November 2010 with its diagnosis established by pathology was analyzed and its clinical manifestations,diagnosis,treatment,and prognosis were discussed according to its domestic and foreign literature.Results The patient’s clinical manifestations included diabetes mellitus,impaired renal function,enlargement of lymph nodes in the neck and groin,high IgG and IgG4 level,and enlargement of pancreas.Abdominal CT showed enlargement of pancreas and interstitial pneumonia.Biopsy of lymph nodes revealed infiltration of IgG4-positive plasma cells.Biopsy of kidney tissue displayed focal sclerosing glomerulonephritis.The renal function became normal and the size of pancreas and lymph nodes was decreased 3 weeks after treatment with corticosteroids.The serum IgG and IgE levels returned to normal and diabetes mellitus was well controlled 20 weeks after treatment with corticosteroids.Conclusion IgG4-related sclerosing disease is a very rare systemic disease,which can be controlled with prompt use of corticosteroids.

关 键 词：IgG4相关硬化性疾病 自身免疫性胰腺炎 IgG4相关间质性肺炎 IgG4相关淋巴结病 

分 类 号：R59[医药卫生—内科学]