Kalra V, Harkiss G D, Lachmann P J, Ghai O P
Clin Exp Immunol. 1982 Oct;50(1):1-6.
Considerable immunological dysfunction has been reported in Indian childhood cirrhosis (Chandra, 1970; Chawla et al., 1973). These observations suggest that the progressive tissue damage observed in this disease may have an immunological basis. To further test this hypothesis, sera from 18 patients with Indian childhood cirrhosis (ICC) and nine age-matched siblings were examined for the presence of soluble immune complexes and activation of complement. The presence of circulating immune complexes (CIC) in ICC is reported for the first time. Immune complexes were measured by the liquid phase 125I-Clq binding (Clq BA) and 125I-conglutinin binding (KBA) assays, and observed in 13/18 (72%) and 10/18 (56%) patients respectively. A significant correlation was found between the two tests. In contrast, only two out of nine siblings had elevated levels of immune complexes, and these were positive in both tests. Evidence was also obtained for in vivo activation of complement via the classical pathway. Total haemolytic complement (CH50) values were depressed in 13/18 (72%) patients, while C4 and C3 values were low in 5/16 (31%) and 4/18 (22%) patients respectively. C3 degradation products were found in substantial amounts in six patients. Complement levels in siblings were uniformly normal. These observations suggest that the immune complexes demonstrated may be of phlogistic significance and merit further characterization.
据报道,印度儿童肝硬化患者存在相当严重的免疫功能障碍(钱德拉,1970年;乔拉等人,1973年)。这些观察结果表明,在这种疾病中观察到的进行性组织损伤可能具有免疫学基础。为了进一步验证这一假设,对18例印度儿童肝硬化(ICC)患者和9名年龄匹配的同胞的血清进行了可溶性免疫复合物和补体激活情况的检测。首次报道了ICC患者中存在循环免疫复合物(CIC)。通过液相125I-Clq结合(Clq BA)和125I-胶固素结合(KBA)试验检测免疫复合物,分别在13/18(72%)和10/18(56%)的患者中观察到。两项检测之间发现有显著相关性。相比之下,9名同胞中只有2人免疫复合物水平升高,且两项检测均呈阳性。还获得了补体通过经典途径在体内激活的证据。13/18(72%)的患者总溶血补体(CH50)值降低,而C4和C3值分别在5/16(31%)和4/18(22%)的患者中较低。在6名患者中发现了大量的C3降解产物。同胞的补体水平均正常。这些观察结果表明,所证实的免疫复合物可能具有促炎意义,值得进一步研究。