Jongen P J, Boerbooms A M, Lamers K J, Raes B C, Vierwinden G
Department of Neurology, University Hospital Nijmegen, The Netherlands.
Neurology. 1990 Oct;40(10):1593-6. doi: 10.1212/wnl.40.10.1593.
In extracerebral systemic lupus erythematosus (SLE), the complement system plays a prominent pathogenic role, and decreased serum concentration of the 4th component (C4) is a reliable indicator of systemic disease activity. In diffuse CNS-SLE, however, the pathogenic role of complement is less clear. In 12 patients with active diffuse CNS-SLE presenting with delirium (4), organic personality syndrome (3), or generalized seizures (5), we determined the CSF indexes of the complement components C3, C4, and factor B, and of IgG, IgA, and IgM. There was a significant increase of the C4 index in these patients compared with controls and a significantly higher CSF C4 index in patients with an increased IgM index. We conclude that intrathecal C4 is being produced in diffuse CNS-SLE.
在脑外系统性红斑狼疮（SLE）中，补体系统起着重要的致病作用，血清中第四成分（C4）浓度降低是系统性疾病活动的可靠指标。然而，在弥漫性中枢神经系统SLE中，补体的致病作用尚不清楚。我们测定了12例伴有谵妄（4例）、器质性人格综合征（3例）或全身性癫痫发作（5例）的活动性弥漫性中枢神经系统SLE患者脑脊液中补体成分C3、C4和B因子以及IgG、IgA和IgM的指标。与对照组相比，这些患者的C4指标显著升高，IgM指标升高的患者脑脊液C4指标显著更高。我们得出结论，弥漫性中枢神经系统SLE中存在鞘内C4生成。
