Wands J R, Dienstag J L, Bhan A K, Feller E R, Isselbacher K J
N Engl J Med. 1978 Feb 2;298(5):233-7. doi: 10.1056/NEJM197802022980502.
We evaluated 20 patients with primary biliary cirrhosis and seven controls with extrahepatic biliary obstruction for presence of circulating immune complexes, having found serologic evidence of alternate complement-pathway activation in eight of the 20. Immune complexes were isolated by cryoprecipitation from serum and measured directly by the sensitive Raji-cell radioimmunoassay. Cryoproteins, found in high concentrations in 90 per cent of the patients with cirrhosis but undetectable in the controls, were composed of IgM (60 per cent), IgG-IgM (25 per cent) and IgA-IgM (5 per cent) and were capable of activating the complement system in vitro. Immune complexes detected by the Raji assay were found in 95 per cent of the patients with cirrhosis and circulated in exceedingly high concentrations (474 microgram per milliliter; range, 16.2 to 2192) but were absent in the controls. Furthermore, the alternate complement pathway was activated in eight cirrhotic patients. These complement-fixing immune complexes differ from immune complexes isolated from other types of liver diseases and may be important in the pathogenesis of primary biliary cirrhosis.
我们评估了20例原发性胆汁性肝硬化患者和7例肝外胆管梗阻对照者循环免疫复合物的存在情况，发现20例患者中有8例存在替代补体途径激活的血清学证据。通过冷沉淀从血清中分离免疫复合物，并采用灵敏的拉吉细胞放射免疫测定法直接进行检测。冷球蛋白在90%的肝硬化患者中浓度很高，但在对照者中未检测到，其由IgM（60%）、IgG-IgM（25%）和IgA-IgM（5%）组成，并且能够在体外激活补体系统。通过拉吉试验检测到的免疫复合物在95%的肝硬化患者中存在，且以极高的浓度（474微克/毫升；范围为16.2至2192）循环，但在对照者中不存在。此外，8例肝硬化患者的替代补体途径被激活。这些补体结合免疫复合物不同于从其他类型肝病中分离出的免疫复合物，可能在原发性胆汁性肝硬化的发病机制中起重要作用。
