Maillet F, Goetz J, Hauptmann G, Clauvel J P, Mery J P, Kahn M F, Kazatchkine M
Presse Med. 1987 Mar 7;16(8):378-82.
Measurements of complement components in sera from patients with systemic lupus erythematosus (SLE) and some of their relatives indicated that decreased levels of CH50, C4 and C2 were mostly related to a genetic deficiency at one or both of the loci coding for C4, at least in those patients in whom decreased C4 levels were associated with normal C1 hemolytic activity. C4 deficiency is either isolated or associated with complement activation. In some patients with C4 deficiency, complement activation could only be demonstrated by measuring plasma level of the C3 cleavage fragment, C3a des Arg. Decreased concentration and/or hemolytic activity of C4 and C2 in SLE cannot be used to assess the activity of the disease.
对系统性红斑狼疮(SLE)患者及其部分亲属血清中的补体成分进行检测,结果表明,CH50、C4和C2水平降低主要与编码C4的一个或两个基因座的遗传缺陷有关,至少在那些C4水平降低与C1溶血活性正常相关的患者中是如此。C4缺乏可为孤立性或与补体激活相关。在一些C4缺乏的患者中,补体激活只能通过检测血浆中C3裂解片段C3a des Arg的水平来证实。SLE患者中C4和C2浓度降低和/或溶血活性降低不能用于评估疾病的活动度。