特发性腹膜后纤维化IgG4的表达及观察  

IgG4 expression in idiopathic retroperitoneal fibrosis

作　　者：管星庆 阚云珍[2] 柳菊[3] 纪元[3] 陈伶俐[3] 谭云山[3] 

机构地区：[1]新县人民医院病理科,河南新县465550 [2]河南省人民医院病理科,郑州450003 [3]复旦大学附属中山医院病理科,上海200032

出　　处：《医药论坛杂志》2015年第3期57-61,共5页Journal of Medical Forum

摘　　要：目的分析特发性腹膜后纤维化的临床病理特点及IgG4的表达,以利于诊断及临床治疗。方法回顾性分析6例诊断特发性腹膜后纤维化病例的临床资料,病理特点。结果 6例特发性腹膜后纤维化患者男女之比4∶2,年龄分别为48-64岁,平均57.5岁。6例均有不同程度腹部胀痛;3例有肾积水;1例伴发热;2例同时伴有其他部位病变。6例血清IgG均升高,IgG4其中4例升高（〉1.35g/L）;5例CRP不同程度升高;6例ESR均升高。CT示腹膜后肿块,包绕大血管,输尿管及肾旁组织等。病理特点是增生的纤维组织中见大量浆细胞,中等量淋巴细胞,并浸润小血管管壁及周隙和神经纤维束周围。免疫组化6例IgG 4＋浆细胞为30-67/HPF,IgG4＋/IgG＋〉40%。结论特发性腹膜后纤维化是Ig G4相关硬化性疾病,确诊需结合临床及实验室检查和影像学,后应排除身体其他部位是否有IgG4相关硬化性疾病,类固醇激素治疗较敏感。Objective To analyze the clinical pathology and IgG4 expression of idiopathic retroperitoneal fibrosis. Method Clinical data and pathological features of 6 cases of idiopathic retroperitoneal were retrospectively analyzed. Results Of the 6 patients ,4 were male and 2 were female, age ranged from 48 - 64 years. All the patients suffered from varying degrees of abdominal pain, 3 patients presented with hydronephrosis, 1 patient had fever, 2 patients accompanied with same lesions in other parts. Serum IgG and ESR were elevated in all the patients while serum IgCA was elevated in 4 （ 〉 1.35g/L） and CRP in 5. CT showed a retroperitoneal mass surrounding the large blood vessels, ureter and kidney and adjacent tissues. Histopathology, they were characterized by proliferation of fibrous tissue infiltrating with a large number of plasma cells, moderate amount of lymphocytes, surrounding tile perivaseular spaces and the nerve fiber bundles and obstructive phlebitis. Immunohistochemistry showed that IgG4 positive plasma cells of 6 cases ranged from 30 to 67/HPF, IgG4 ＋/IgG ＋ 〉 40%. Conclusion Idiopathic retroperitoneal fibrosis is a IgCA - related sclerosing disease, its diagnosis requires a combination of clinical,laboratory tests and imaging. Once the diagnosis is made, other parts of the body of IgG4 - related selerosing disease should be excluded, and closing follow - up should be made. The disease is sensitive to steroid therapy.

关 键 词：腹膜后纤维化 IGG4相关性疾病 IGG4 鉴别诊断 

分 类 号：R656.4[医药卫生—外科学]