Ann Intern Med. 1979 Jan;90(1):72-84. doi: 10.7326/0003-4819-90-1-72.
Primary biliary cirrhosis is a disease characterized by slowly progressive intrahepatic cholestasis, destructive lesions of the septal and larger interlobular bile ducts, and granulomas. It is associated with defects of both humoral and cellular immune function. As part of the detailed evaluation of these defects, the status of the complement system has been evaluated. Striking abnormalities of serum complement levels are found but are difficult to interpret. However, the demonstration of marked hypercatabolism of C3, but not albumin, suggests that the complement system may be in a chronically activated state. Furthermore, an unequivocal defect in the clearance of sensitized erythrocytes by receptors for C3b on Kupffer cells has been found. One possible explanation for this finding would be that a large proportion of these receptors are occupied either by immune complexes containing C3b or excess free C3b that is generated by complement activation. Major defects of C3 catabolism and C3b-receptor-mediated clearance are not found in patients with HBsAg-negative chronic active hepatitis. These findings suggest a role for the complement system in the pathophysiology of primary biliary cirrhosis.
原发性胆汁性肝硬化是一种以缓慢进展的肝内胆汁淤积、间隔和较大小叶间胆管的破坏性病变以及肉芽肿为特征的疾病。它与体液免疫和细胞免疫功能缺陷均有关。作为对这些缺陷进行详细评估的一部分，已经对补体系统的状态进行了评估。发现血清补体水平存在显著异常，但难以解释。然而，C3出现明显的高分解代谢，而白蛋白则没有，这表明补体系统可能处于慢性激活状态。此外，还发现枯否细胞上C3b受体清除致敏红细胞存在明确缺陷。对此发现的一种可能解释是，这些受体的很大一部分被含有C3b的免疫复合物或补体激活产生的过量游离C3b占据。在HBsAg阴性的慢性活动性肝炎患者中未发现C3分解代谢和C3b受体介导的清除存在主要缺陷。这些发现提示补体系统在原发性胆汁性肝硬化的病理生理学中起作用。
