免疫球蛋白G4相关性硬化性胆管炎（附七例病例分析）  

Immunoglobilin G4-related sclerosing cholangitis （7 cases report）

作　　者：沈盛[1] 刘厚宝[1] 锁涛[1] 刘寒[1] 王越琦[1] 

机构地区：[1]复旦大学附属中山医院普外科,上海200032

出　　处：《腹部外科》2015年第5期305-309,F0002,共6页Journal of Abdominal Surgery

基　　金：复旦大学附属中山医院青年基金资助

摘　　要：目的 免疫球蛋白G4相关性硬化性胆管炎（IgG4-related sclerosing cholanitis,ISC）是一类特殊的硬化性胆管炎,目前国内外对其的认识仍远远不足.因此希望通过回顾性分析总结以提高对该疾病的认识水平.方法 回顾性分析近4年来所收治ISC病例的临床特点.结果 4年间共收治ISC病例7例,多为老年（平均年龄65.4岁）男性（85.7％）,均以轻度黄疸起病（平均总胆红素为58.0 μmol/L）,多数病例血清IgG4明显升高（85.7％,平均IgG4值为11 g/L）,肿瘤标志物多在正常范围（85.7％）,自身抗体均正常;6例位于肝门部胆管,1例首先位于胰腺段胆管后累及肝总管和右肝内胆管;其中有3例难以明确诊断予以手术探查,术后病理学检查明确;最终7例病例均接受激素治疗并长期随访,病情好转明显.结论 对于所有胆管狭窄的病例均应怀疑ISC的可能,同时需警惕恶性肿瘤的可能性,常规检测IgG4和自身抗体以及仅轻度黄疸有助于鉴别诊断,而对于难以明确者可考虑病理学检查明确,多数ISC对于激素治疗敏感.Objective Immunoglobulin G4-related sclerosing cholangitis （ISC）, which was rec- ognized as a new clinicopathological entity in cholangitis, has been identified worldwide. A series of exploration were carried out for the pathogenesis and differential diagnosis, however, the recognition of this disease is still less. The purpose of this study is to provide insight into the disease and may be helpful to clinical work. Methods Included in this single-center retrospective clinical study were 7 patients with the diagnosis of ISC between June 2011 and May 2015. Results ISC patients generally were older （mean age, 65.4 years） men （85.7%）, presenting with mild obstructive jaundice （100%, mean total bilirubin, 58. 0/zmol/L）, increased serum IgG4 levels （85.7%, mean IgG4, 11 g/L）, tumor marks （85.7%） and autoantibody in normal （100%）. Biliary strictures were confined to the hilar bile duct （85.7%） and distal bile duct （14. 3%）. The definite diagnosis was made in 3 patients after surgery because of suspected malignant tumor. All patients received steroid therapy and well followed up. Conclusions Biliary strictures should be all suspected of ISC, and mild jaundice, routine serum IgG4 and autoantibody test are helpful to identify ISC. Correct diagnosis will avoid unneccessary surgery because ISC responds well to steroid therapy.

关 键 词：免疫球蛋白G4相关性硬化性胆管炎 胆管癌 硬化性胆管炎 

分 类 号：R657.45[医药卫生—外科学]