IgG4相关硬化性胆管炎的临床特征及影像学表现

Clinical and imaging features of immunoglobulin G4-Related sclerosing cholangitis

目的探讨分析IgG4相关硬化性胆管炎的临床特征和CT及MRI表现,旨在提高对IgG4相关硬化性胆管炎(IAC)诊断及鉴别诊断水平。方法选取经诊断标准及手术病理确诊的22例IAC患者,分析其临床资料、实验室指标及CT和MRI影像学特征。结果IAC主要表现为受累胆管单发或多发环形增厚,易发生于胆总管胰腺段,T 2WI呈稍低或等信号,增强后持续性明显强化,磁共振胰胆管成像(MRCP)显示远端胆管轻度扩张,呈“枯枝”征;淋巴结肿大少见,较多累及胰腺、胆囊、肾脏及腹膜后等器官;IAC患者通过激素及免疫抑制剂治疗均可得到缓解及治愈。结论IAC的影像学表现,特别是MRI表现具有一定特征,结合临床特征能够有效提高鉴别诊断能力,为临床治疗方案的制定提供重要依据。

Objective To analyse clinical and imaging features of immunoglobulin G4-related sclerosing cholangitis(IAC),in order to improve the level of diagnosis and differential diagnosis.Methods The clinical data,laboratory index and CT and MRI features of 22 patients with IAC confirmed by diagnostic criteria and pathology were retrospectively analyzed.Results The manifestations of IAC were annular thickening of bile duct wall,mainly occurs on choledochal duct at pancreas level,low or equal signal in T_(2)WI,significant and persistent enhancement in the arterial,venal and delayed phase.MRCP showed only mild dilatation of bile duct like“deadwood sign”.Lymphadenectasis was rare,IAC involved easily pancreas,gallbladder,kidney and retroperitoneum.IAC disease could be relieved and cured after corticosteroids and immunosuppressive agent treatment.Conclusion IAC has the typical features of the imaging especially MRI,combining the clinical and laboratory indexes can improve the diagnosis and differential diagnosis of IAC and provide valuable information for clinical treatment planning.