IgG4相关性硬化性胆管炎100例临床特征分析

doi:10.3969/j.issn.1672-5069.2021.01.022

摘要/Abstract

摘要： 目的由于临床和影像学表现的相似及临床医生认知不足,IgG4相关性硬化性胆管炎(IgG4-SC)常被误诊为原发性硬化性胆管炎(PSC)或胆管癌(CC)等胆汁淤积性疾病,而采取了不必要的手术治疗,因此对IgG4-SC的准确认识非常重要。目前,对IgG4-SC的临床研究比较缺乏,文献多为零星的临床病例报道,缺乏系统总结。我们通过汇总分析国内报道的病例,探讨了IgG4-SC患者的临床特点和误诊原因,以提高对该病的认识和诊断水平。方法以“IgG4相关性硬化性胆管炎”为关键词在中国知网数据库、万方数据库和维普数据库检索2019年9月前公开发表的文献,并提取相关病例的一般资料、临床表现、实验室检查、影像学表现、病理学特征、误诊情况、治疗和预后等信息进行汇总分析。结果纳入25篇文献,包括100例患者,男女比例为4∶1,平均发病年龄为(58.8±12.3)岁;最常见的临床表现为黄疸(85%)和腹痛(53%); 94.2%(81/86)患者血清IgG4水平增高,33%患者血清CA19-9升高;影像学表现为胆总管下段狭窄57例,肝门区胆管狭窄伴肝内胆管扩张15例;34例肝组织检查发现胆囊壁/胆管壁纤维组织增生伴大量淋巴细胞和浆细胞浸润,可见闭塞性静脉炎或席纹状硬化;免疫组化检测,15例显示IgG4⁺浆细胞>10/HPF;在100例中,12例因误诊为胆管癌而实施手术治疗,61例应用糖皮质激素治疗,58例(95%)病情缓解。结论 IgG4-SC患者多以梗阻性黄疸和腹痛为主要表现,在临床和影像学表现上与原发性硬化性胆管炎和胆管癌十分相似,需注意鉴别诊断。

关键词: IgG4相关性硬化性胆管炎, 临床特征, 汇总分析

Abstract: Objective Due to the similarity of clinical and imaging manifestations and lack of awareness of this disease, IgG4-related sclerosing cholangitis (IgG4-SC) is often misdiagnosed as primary sclerosing cholangitis (PSC), cholangiocarcinoma (CC) or other cholestatic diseases, and unnecessary surgical treatment is performed. The accurate understanding of IgG4-SC is very important. However, available clinical studies about IgG4-SC are comparatively insufficient, and most literatures are reports of sporadic cases, there is a need of systematic review of the entity. We need to know the clinical characteristics and misdiagnosis causes of IgG4-SC, and to improve the clinical diagnosis. Methods With "IgG4-related sclerosing cholangitis" as the key word, the literature published before September 2019 was searched on CNKI database, WANFANG database and VIP database, including general information, clinical manifestations, laboratory examinations, imaging findings, pathological features, misdiagnosis, treatment and prognosis of relevant cases. Results A total of 25 articles, including 100 patients, were obtained. The sex ratio of them was 4:1, with the mean age of illness onset of (58.8±12.3) years; the most common symptoms were jaundice (85%, 85/100) and abdominal pain (53%, 53/100); the serological tests showed that serum IgG4 level increased in 94.2% (81/86) of patients, and serum CA19-9 level increased in 33% of patients; the imaging findings included bile duct stenosis in the lower common bile duct in 57 cases and bile duct stricture in the hilar region with intrahepatic bile duct dilatation in 15 cases; the pathological findings in 34 cases examined showed the hyperplasia of fibrous tissue in the wall of gallbladder/bile duct with numerous lymphocyte, plasma cell infiltration, and obliterative phlebitis or storiform fibrosis; the immunohistochemistry in 15 cases showed the IgG4⁺ plasma cell were >10/HPF; out of the 100 patients, 12 cases were misdiagnosed as CC and surgical operation were inappropriately underwent, and 61 cases were treated with glucocorticoids, with remission in 58 cases (95%).Conclusion The main manifestations of patients with IgG4-SC are obstructive jaundice and abdominal pain, which are very similar to primary sclerosing cholangitis, cholangiocarcinoma, pancreatic cancer and other diseases, so it is necessary to pay attention to the differential diagnosis in this setting.

Key words: IgG4-related sclerosing cholangitis, Clinical features, Systemic analysis

孙文霞, 伍树芝. IgG4相关性硬化性胆管炎100例临床特征分析[J]. 实用肝脏病杂志, 2021, 24(1): 83-86.

Sun Wenxia, Wu Shuzhi. Clinical features of IgG4-related sclerosing cholangitis:systemic literature review of 100 cases[J]. Journal of Practical Hepatology, 2021, 24(1): 83-86.

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