Nakanuma Y, Kato Y, Unoura M, Kobayashi K, Yamamoto M
Second Department of Pathology, Kanazawa University School of Medicine, Japan.
Acta Pathol Jpn. 1988 Feb;38(2):249-57. doi: 10.1111/j.1440-1827.1988.tb01103.x.
A rare autopsy case of primary sclerosing cholangitis with sequential histologic observations of the liver is described. The patient, a 62-year-old female at the time of autopsy, presented with prolonged cholestasis of about 9 years duration. Initial (at 53 years) and second (at 59 years) liver biopsies disclosed fibrous enlargement of the portal tracts with loss of interlobular bile ducts, lymphoplasmacytic infiltration, a few epithelioid granulomas, piecemeal necrosis, atypical ductular proliferation and deposition of copper granules. Hypergammaglobulinemia with elevated IgM was also noted. These clinicopathological features resembled primary biliary cirrhosis. However, no florid duct lesions were found, and absence of antimitochondrial antibodies and cholangiographic demonstration of a beaded biliary tree favored a diagnosis of primary sclerosing cholangitis. The autopsied liver disclosed sclerosis and cholangioectases of the intra- and extrahepatic biliary tree in addition to biliary cirrhosis. The histology of the biliary tree disclosed nonspecific fibrosing inflammation in the extra- and intrahepatic biliary tree. Other autopsy findings included chronic thyroiditis, sialoadenitis and pancreatitis.
本文描述了一例罕见的原发性硬化性胆管炎尸检病例，并对肝脏进行了连续的组织学观察。该患者在尸检时为一名62岁女性，出现了约9年的长期胆汁淤积。初次（53岁时）和二次（59岁时）肝脏活检显示门静脉区纤维性增大，小叶间胆管缺失，淋巴浆细胞浸润，少量上皮样肉芽肿，碎片状坏死，非典型小胆管增生以及铜颗粒沉积。还发现有高球蛋白血症伴IgM升高。这些临床病理特征类似于原发性胆汁性肝硬化。然而，未发现典型的胆管病变，且无抗线粒体抗体以及胆管造影显示串珠状胆管树，这些均支持原发性硬化性胆管炎的诊断。尸检肝脏除了有胆汁性肝硬化外，还显示肝内和肝外胆管树的硬化和胆管扩张。胆管树的组织学检查显示肝内和肝外胆管树存在非特异性纤维性炎症。其他尸检发现包括慢性甲状腺炎、涎腺炎和胰腺炎。
