误诊为胆管肿瘤的两例IgG4相关性胆管炎的诊治分析

Analysis of two cases of IgG4 associated cholangitis which were misdiagnosed as biliary tumor

目的 通过分析两例术前误诊为胆管肿瘤的IgG4相关性胆管炎的诊治经验和教训,初步探讨IgG4相关性胆管炎疾病的诊断要点和治疗策略.方法 总结2013年3月-2013年4月在南京大学医学院附属鼓楼医院肝胆外科就诊的2例IgG4相关性胆管炎的临床资料,包括病史、体征、实验室检查.重点回顾手术探查、术式选择、术后病理及免疫学指标以及对诊断治疗的意义.结果 2例术前的影像学检查均高度怀疑高位胆管肿瘤.其中1例因病灶位于右肝管而以胆管癌根治的标准行右半肝切除,及高位胆肠吻合术,术后病理诊断为IgG4相关性胆管炎.另1例病灶位于肝总管,行肿块局部切除术,术后病理及免疫学指标均提示为IgG4相关性胆管炎.术后恢复顺利,痊愈出院.结论 IgG4相关性胆管炎是较为罕见的自身免疫性疾病,重点需要与胆管癌鉴别诊断,手术治疗具有临床意义,激素应用在术后恢复治疗中起到一定的作用.

Objective To analyze the two cases of IgG4 associated cholangitis which were misdiagnosed as biliary tumor,and explore the diagnostic criteria and treatment strategies of IgG4-related cholangitis.Methods Summary the clinical data of two cases of IgG4-related cholangitis from Mar to Apr.2013 in Hepatopancreatobiliary Surgery Department of Drum Tower Hospital Affiliated to Nanjing University Medical School,including medical history,signs and laboratory examinations.Focusing on reviewing the surgical exploration,choice of surgical methods,postoperative pathologies and immunology indexes as well as their significance for diagnosis and treatments.Results Two cases of preoperative imaging studies were highly suggestive of Hilar cholangiocarcinoma.For lesions located in the right hepatic duct,one case was subjected to right hepatectomy and high bilioenteric anastomosis according to therapy standard of cholangiocarcinoma,but the pathological diagnosis revealed IgG4-related cholangitis; Another case was underwent local resection,for the lesion located in the hepatic duct,the pathology and immunology indexes were suggestive of IgG4-related cholangitis.The postoperative recovery was good,and was discharged well.Conclusions IgG4 related cholangitis is a rare autoimmune disease,which needs to priority differential diagnosis of cholangiocarcinoma.Operation plays an important role in its treatment,and hormone is helpful.