Hereditary C2 deficiency and systemic lupus erythematosus associated with severe glomerulonephritis.
Hereditary C2 deficiency and systemic lupus erythematosus associated with severe glomerulonephritis.
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遗传性 C2 缺乏症和系统性红斑狼疮与严重肾小球肾炎相关。
DOI：
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发表时间：
1978
影响因子：
4.6
通讯作者：
E. Lewis
中科院分区：
文献类型：
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作者：
J. Roberts;M. Schwartz;E. Lewis
尽管在遗传性C2缺乏症患者中有异常高的免疫疾病发病率，但这些疾病的严重程度相对较轻，这表明阻断补体激活C4以外的补体可以防止补体介导的炎症。本报告描述了一例伴有严重系统性红斑狼疮（SLE）和弥漫性增生性肾小球肾炎的c2缺陷患者的研究。肾的免疫病理学研究显示properdin、properdin因子B、C3和C5的沉积模式与免疫球蛋白G沉积相似。在患者病程的不同时期，血清适宜素和适宜素因子B水平均较低。体外补体固定研究表明，肾小球沉积物的C3固定可以通过替代途径发生。对患者皮肤中的免疫沉积物的研究也得出了类似的结果。这些研究表明，炎症可能通过替代补体途径在C2缺陷-狼疮综合征中有效介导。
Although an unusually high incidence of immunological diseases has been described in patients with hereditary C2 deficiency, the severity of these illnesses has been relatively mild, suggesting that blocking complement activation beyond C4 may protect against significant complement-mediated inflammation. This report describes studies in a C2-deficient patient with severe systemic lupus erythematosus (SLE) and diffuse proliferative glomerulonephritis. An immunopathological study of the kidney revealed the deposition of properdin, properdin factor B, C3 and C5 in a pattern similar to immunoglobulin G deposits. Serum properdin and properdin factor B levels were low at various times during the patient's course. In vitro complement fixation studies showed C3 fixation by glomerular deposits could occur via the alternative pathway. Studies of the immune deposits in the patients' skin revealed similar results. These studies suggest that inflammation may be effectively mediated via the alternative complement pathway in the C2 deficiency-lupus syndrome.