| 高天宇,李建军.IgG4相关性硬化性胆管炎诊疗进展[J].济宁医学院学报,2020,43(1):63-67 |
| IgG4相关性硬化性胆管炎诊疗进展 |
| Progress in the diagnosis and treatment of IgG-4 related sclerosing cholangitis |
| 投稿时间：2020-01-09 |
| DOI：10.3969/j.issn.1000-9760.2020.01.015 |
| 中文关键词: 免疫球蛋白G;硬化性胆管炎;胆管癌 |
| 英文关键词: Immunoglobulin G;Sclerosing cholangitis;Cholangiocarcinoma |
| 基金项目: |
| 作者 | 单位 | E-mail | | 高天宇 | 济宁医学院临床医学院, 济宁 272013
济宁医学院, 济宁 272067 | | | 李建军 | 济宁医学院临床医学院, 济宁 272013
济宁医学院, 济宁 272067 | lixv16@sohu.com |
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| 中文摘要: |
| IgG4相关性硬化性胆管炎（IgG4-SC）是由IgG4阳性浆细胞、淋巴细胞浸润和胆管壁纤维化所导致的胆管进行性狭窄和破坏的一种疾病，主要表现为：胆汁淤积酶水平持续升高；血清IgG4水平显著升高；肝内外胆管弥漫性或节段性狭窄；组织学中淋巴浆细胞和IgG4阳性浆细胞浸润；IgG4相关的并存疾病，特别是自身免疫性胰腺炎；对类固醇有较好的疗效。IgG4-SC发病率低，临床对其认识不足，IgG4-SC影像学表现与肝门部胆管癌或原发性硬化性胆管炎（PSC）相似，临床误诊时有发生，部分IgG4-SC病例因怀疑胆管癌而行手术切除。本文将对IgG4-SC的流行病学、发病机制、诊断、临床治疗的研究进展作一综述。 |
| 英文摘要: |
| IgG4-related sclerosing cholangitis (IgG4-SC) is a disease of progressive narrowing and destruction of the bile ducts caused by IgG4-positive plasma cells,lymphocyte infiltration,and bile duct wall fibrosis.Its typical clinical characteristics are:chronic elevation of cholestatic enzyme levels;significant elevation of serum IgG4 levels;diffuse or segmental stenosis of the intrahepatic or extrahepatic bile ducts;marked lymphoplasmacytic and IgG4-positive plasma cell infiltration;presence of IgG4-RD in other organs,mainly involving autoimmune pancreatitis;excellent response to corticosteroids.However,the cholangiographic findings of IgG4-SC were similar to those of hilar cholangiocarcinoma or (PSC),and some cases of IgG4-SC underwent surgical resection because of suspected cholangiocarcinoma.Therefore,this article reviews the epidemiology,pathogenesis,diagnosis,and clinical treatment of IgG4-SC. |
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