Popova I V, Beketova T P, Severov M V, Prokhorova M V
Arkh Patol. 1993 May-Jun;55(3):21-5.
9 cases (males) of primary sclerosing cholangitis (PSC) were studied clinico-morphologically. In puncture liver biopsies, sclerosis of walls of big ducts and portal tracts was found in 2 cases only; cell infiltration was minimal. Lobular hepatitis with intralobular fibrosis and moderate sclerosis of portal stroma was found in 3 patients, monolobular inactive liver cirrhosis in 4 patients. In all cases mild intralobular cholestasis was found which was followed with a considerable increase of copper and manganese in the liver and serum. The accumulation of a large amount of collagen of different types was observed around bile ducts which ultrastructurally was seen as fibrillar structures and homogeneous substance similar to the basal membrane. The elements of the epithelium destruction were found in foci of the duct basal membrane alteration, in the absence of cell infiltration. Progressing fibrosis of both portal tracts without inflammation and individual segments of the bile ducts and the intralobular stroma is at the basis of PSC.
对9例（男性）原发性硬化性胆管炎（PSC）患者进行了临床形态学研究。在经皮肝穿刺活检中，仅2例发现大胆管壁和门管区硬化；细胞浸润轻微。3例患者出现小叶性肝炎伴小叶内纤维化和门管间质中度硬化，4例患者出现单小叶性静止性肝硬化。所有病例均发现轻度小叶内胆汁淤积，随后肝脏和血清中的铜和锰显著增加。在胆管周围观察到大量不同类型胶原蛋白的积累，超微结构上表现为纤维状结构和类似于基底膜的均质物质。在胆管基底膜改变的病灶中发现上皮破坏的成分，无细胞浸润。PSC的基础是无炎症的门管区以及胆管和小叶内间质的个别节段进行性纤维化。
