Tidsskr Nor Laegeforen. 2020 Feb 10;140(3). doi: 10.4045/tidsskr.19.0390. Print 2020 Feb 25.
Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease which can potentially affect any organ system. IgG4-related sclerosing cholangitis and inflammatory pseudotumour in the hepatobiliary system is rare, but is probably underdiagnosed.
We present the case of a 52-year-old male who was admitted with obstructive jaundice and weight loss. He presented with a mass lesion in the porta hepatis mimicking hilar cholangiocarcinoma. The patient underwent extended right hepatectomy with hepaticojejunostomy. Severe liver failure developed postoperatively, and the patient underwent liver transplantation. The resected specimen showed infiltration of IgG4 positive plasma cells in the liver hilum, and immunohistochemical staining demonstrated a ratio of IgG4/IgG-positive plasma cells of more than 40 %. Postoperative serological testing showed elevated levels of serum IgG4 6.0 g/L (0.03-2.01), and the CT imaging revealed chronic pancreatitis and bilateral enlargement of the submandibular glands. The patient was ultimately diagnosed with IgG4-related disease.
It is difficult to distinguish benign bile duct strictures in the porta hepatis from hilar cholangiocarcinoma, and serum IgG4 is unreliable as a diagnostic marker due to low sensitivity and specificity. Greater awareness of IgG4-RD is needed in order to avoid surgery.
免疫球蛋白 G4 相关疾病（IgG4-RD）是一种可能影响任何器官系统的全身性疾病。IgG4 相关硬化性胆管炎和肝胆系统炎症性假瘤较为罕见，但可能诊断不足。
我们报告了一例 52 岁男性，因梗阻性黄疸和体重减轻入院。患者肝门区有一肿块，类似于肝门部胆管癌。患者接受了扩大右半肝切除术和胆肠吻合术。术后出现严重肝功能衰竭，患者接受了肝移植。切除标本显示肝门处有 IgG4 阳性浆细胞浸润，免疫组化染色显示 IgG4/IgG 阳性浆细胞比例大于 40%。术后血清学检查显示血清 IgG4 水平升高至 6.0 g/L（0.03-2.01），CT 影像学显示慢性胰腺炎和双侧下颌下腺肿大。最终诊断为 IgG4 相关疾病。
肝门部良性胆管狭窄与肝门部胆管癌难以区分，血清 IgG4 作为诊断标志物的敏感性和特异性均较低，不可靠。需要提高对 IgG4-RD 的认识，以避免手术。
