Ikeda H, Oka T, Imafuku I, Yamada S, Yamada H, Fujiwara K, Hirata M, Idezuki Y, Oka H
First Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Japan.
Am J Gastroenterol. 1990 Feb;85(2):203-6.
A rare cause of obstructive jaundice is presented. A 43-yr-old man developed jaundice with fever and weight loss, and showed lymphadenopathy, reticular shadows on chest roentgenogram, deranged liver function tests, eosinophilia, low values of complement proteins, and hypergammaglobulinemia. There was a stricture throughout the bilateral hepatic ducts and common hepatic and bile ducts with dilated intrahepatic bile ducts on imagings. The walls of the gallbladder and common bile duct were thickened due to diffuse granulomatous lesions of unknown etiology, composed mainly of plasma cells, lymphocytes, and fibroblasts identical with inflammatory pseudotumor. The same histological findings also were observed in the lung and lymph nodes. Jaundice disappeared, with reduced thickening of the common bile duct wall. All clinical and laboratory abnormalities subsided after high-dose prednisolone therapy, starting when dyspnea developed. There is no similar case in the literature.
本文报道了一例梗阻性黄疸的罕见病因。一名43岁男性出现黄疸伴发热和体重减轻,有淋巴结肿大、胸部X线片显示网状阴影、肝功能检查异常、嗜酸性粒细胞增多、补体蛋白值降低及高球蛋白血症。影像学检查显示双侧肝管、肝总管和胆总管全程狭窄,肝内胆管扩张。胆囊壁和胆总管壁因病因不明的弥漫性肉芽肿性病变而增厚,主要由与炎性假瘤相同的浆细胞、淋巴细胞和成纤维细胞组成。在肺和淋巴结中也观察到相同的组织学表现。黄疸消失,胆总管壁增厚减轻。自出现呼吸困难开始给予大剂量泼尼松龙治疗后,所有临床和实验室异常均消退。文献中无类似病例。