在一名C2缺陷型系统性红斑狼疮患者血清中观察到的残余免疫复合物红细胞结合活性中，B因子的参与可能会延迟临床症状的出现。
Participation of factor B in residual immune complex red cell binding activity observed in serum from a C2-deficient systemic lupus erythematosus patient may delay the appearance of clinical symptoms.
作者信息
Traustadóttir K H, Rafnar B O, Steinsson K, Valdimarsson H, Erlendsson K
机构信息
The National University Hospital, Landspítalinn, Reykjavik, Iceland.
出版信息
Arthritis Rheum. 1998 Mar;41(3):427-34. doi: 10.1002/1529-0131(199803)41:3<427::AID-ART8>3.0.CO;2-7.
OBJECTIVE
To investigate whether participation of factor B (FB) in immune complex transport might explain long periods of clinical remissions in a homozygous C2-deficient patient with systemic lupus erythematosus (SLE) treated regularly with plasma infusions.
METHODS
Immune complex red cell binding (ICRB) was assayed as enzyme activity, C3d by enzyme-linked immunosorbent assay, and FB by immunoelectrophoresis.
RESULTS
C2-deficient sera showed low-grade ICRB, which correlated with levels of FB. This activity could be blocked with antibodies to C1q, C4, or FB, but not by antibodies to C2. C3d levels in the patient's plasma changed during infusion, followed by a gradient increase during remission. Comparison of ICRB, C3d, and FB suggested an inverse relationship between FB levels and clinical symptoms.
CONCLUSION
In C2 deficiency, FB may interact with C4 to provide a low-grade ICRB. This activity could be clinically significant in patients with C2 deficiency and explain why they are less prone to SLE than patients with C1q or C4 deficiency.
目的
研究B因子（FB）参与免疫复合物转运是否可以解释定期接受血浆输注治疗的纯合子C2缺陷型系统性红斑狼疮（SLE）患者出现长时间临床缓解的原因。
方法
采用酶活性法检测免疫复合物红细胞结合（ICRB），用酶联免疫吸附测定法检测C3d，用免疫电泳法检测FB。
结果
C2缺陷血清显示低水平的ICRB，这与FB水平相关。该活性可被抗C1q、C4或FB的抗体阻断，但不能被抗C2的抗体阻断。患者血浆中的C3d水平在输注期间发生变化，随后在缓解期呈梯度升高。ICRB、C3d和FB的比较表明FB水平与临床症状呈负相关。
结论
在C2缺陷中，FB可能与C4相互作用以提供低水平的ICRB。这种活性在C2缺陷患者中可能具有临床意义，并解释了为什么他们比C1q或C4缺陷患者患SLE的倾向更低。
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