Minh Thong Pham, Dang Luu Vu, Tra My Thieu-Thi, Xuan Hien Nguyen, Anh Tuan Tran, Minh Duc Nguyen
Department of Radiology, Ha Noi Medical University, Hanoi, Vietnam.
Radiology Center, Bach Mai Hospital, Hanoi, Vietnam.
Case Rep Oncol. 2021 Feb 18;14(1):39-46. doi: 10.1159/000513029. eCollection 2021 Jan-Apr.
IgG4-related sclerosing cholangitis (IgG4-SC) is a relatively newly identified disease that is frequently associated with autoimmune pancreatitis. The differential diagnosis between cholangiocarcinoma, primary sclerosing cholangitis, and IgG4-SC can be challenging due to significant overlap among the clinical and imaging characteristics. We report the case of a 71-year-old woman who was diagnosed with IgG4-related disease based on increased serum IgG4 levels, imaging, and clinical presentation, which showed systemic involvement, including sclerosing cholangitis and kidneys. The patient presented with chronic jaundice. Magnetic resonance imaging revealed bile duct strictures and the dilatation of upstream bile ducts, smooth wall thickening with uniform enhancement in the delayed phase, and no vascular infiltration. Multiple low-density, wedge-shaped areas were identified in both kidneys, which were hypointense on T2-weighted images and hyperintense on diffusion-weighted images. The serum IgG4 levels of this patient were elevated to nearly 10-fold the normal upper limit. A diagnosis of IgG4-SC associated with IgG4-related kidney was made. Based on this case, pre-surgery IgG4 serum treatment in patients with non-malignant bile duct stenosis was recommended to exclude IgG4-SC.
IgG4相关性硬化性胆管炎（IgG4-SC）是一种相对较新发现的疾病，常与自身免疫性胰腺炎相关。由于胆管癌、原发性硬化性胆管炎和IgG4-SC在临床和影像学特征上存在显著重叠，因此它们之间的鉴别诊断具有挑战性。我们报告了一例71岁女性病例，该患者根据血清IgG4水平升高、影像学表现及临床表现被诊断为IgG4相关疾病，临床表现显示全身受累，包括硬化性胆管炎和肾脏。患者表现为慢性黄疸。磁共振成像显示胆管狭窄及上游胆管扩张，管壁光滑增厚，延迟期均匀强化，无血管浸润。双侧肾脏均发现多个低密度楔形区域，在T2加权图像上呈低信号，在扩散加权图像上呈高信号。该患者血清IgG4水平升高至正常上限的近10倍。诊断为IgG4-SC合并IgG4相关性肾病。基于该病例，建议对非恶性胆管狭窄患者在术前进行IgG4血清治疗以排除IgG4-SC。
