Garred P, Mollnes T E, Thorsteinsson L, Erlendsson K, Steinsson K
Institute of Immunology and Rheumatology, Rikshospitalet, National Hospital, Oslo, Norway.
Scand J Immunol. 1990 Jan;31(1):59-64. doi: 10.1111/j.1365-3083.1990.tb02743.x.
Plasma and serum samples from a patient with homozygous C2 deficiency and severe systemic lupus erythematosus who responded with full clinical remission after plasma infusions were examined for immune complexes (IC), C3 activation products, and the terminal complement complex (TCC). Plasma contained large amounts of C4-containing IC but no C3-containing IC or complement activation products. Classical pathway activation in vitro did not lead to C3 activation or TCC formation as seen in normal serum, but a very efficient binding of C1q and C4 was found. No disturbances in alternative pathway activation were observed. The results indicate an impaired formation of C3-containing IC and an inefficient clearance of C4-containing IC, supporting the idea of a causal relationship between the dysfunctional classical pathway, pathophysiology, and clinical manifestations in this patient.
对一名纯合子C2缺陷且患有严重系统性红斑狼疮的患者进行血浆输注后实现完全临床缓解，采集其血浆和血清样本检测免疫复合物（IC）、C3激活产物和末端补体复合物（TCC）。血浆中含有大量含C4的IC，但不含含C3的IC或补体激活产物。体外经典途径激活未像正常血清那样导致C3激活或TCC形成，但发现C1q和C4有非常有效的结合。未观察到替代途径激活存在紊乱。结果表明含C3的IC形成受损以及含C4的IC清除效率低下，支持了该患者经典途径功能失调、病理生理学和临床表现之间存在因果关系的观点。
