疑似恶性肿瘤的IgG4相关硬化性胆管炎5例临床分析

IgG4-related sclerosing cholangitis suspected of being malignant tumors:A case series

背景IgG4相关性硬化性胆管炎(IgG4 related sclerosing cholangitis,IgG4-SC)是IgG4相关性疾病(IgG4-related disease,IRD)的胆道表现.IgG4-SC常有胆管壁局限性增厚、胆管扩张以及梗阻性黄疸等特点,其表现与胆胰恶性肿瘤类似,临床上容易延迟诊断甚至误诊.目的总结IgG4-SC的患者临床特征和诊治过程,提高临床医师对该病的认识,避免误诊.方法回顾性分析了2012年至2020年间入院影像学检查发现胆管炎同时伴有肝门、壶腹、胰腺疑似占位病变的5例IgG4-SC.采集患者基本信息,包括年龄、性别、症状、体征、既往病史、诊疗过程.结果4例表现为梗阻性黄疸,影像学提示胆道高位梗阻1例,低位梗阻3例,梗阻部位位于肝门、胰头部或壶腹部,疑似恶性占位;1例为体检发现胰颈部占位,恶性不除外.实验室检查可见胆红素升高、以直接胆红素为主,伴肝功能损伤,血清IgG4水平明显升高;肿瘤标志物正常或轻度升高.4例患者经多学科会诊确诊为IgG4-SC,1例手术后病理确诊.结论多学科会诊是认识IgG4-SC、避免误诊的重要经验.

BACKGROUND IgG4-related sclerosing cholangitis(IgG4-SC)is the biliary manifestation of IgG4-related disease(IRD).IgG4-SC often has the characteristics of localized thickening of the bile duct wall,dilation of the bile duct,and obstructive jaundice,which are similar to those of biliary and pancreatic malignancies and often lead to delayed diagnosis or even misdiagnosis clinically.AIM To improve the clinicians’understanding of IgG4-SC and avoid its misdiagnosis by analyzing the clinical characteristics,diagnosis,and treatment of five cases of IgG4-SC.METHODS A retrospective analysis was performed on five cases of IgG4-SC suspected of being space-occupying lesions of the porta hepatis,ampulla,and pancreas at admission.Patient information,including age,gender,symptoms,signs,past medical history,and the process of diagnosis and treatment,was collected.RESULTS Four cases showed obstructive jaundice and imaging identified one case of high level biliary obstruction and three cases of low level obstruction,which suggested the possibility of malignancy.The obstruction sites were located in the porta hepatis,pancreatic head,or ampulla.One case was found to have a mass at the neck of the pancreas by physical examination.All five patients had bile duct inflammation,swelling of the pancreas,or space-occupying lesions in the imaging examination.Laboratory examinations revealed elevated bilirubin,mainly direct bilirubin,accompanied by liver dysfunction.Serum IgG4 levels were significantly increased and tumor markers were normal or slightly elevated.Four patients were diagnosed with IgG4-SC by multidisciplinary consultation,and one patient was diagnosed by pathological examination after surgery.CONCLUSION Multidisciplinary consultation is an important strategy in diagnosing IgG4-SC and avoiding misdiagnosis.