Ng Y C, Walport M J
Baillieres Clin Rheumatol. 1988 Dec;2(3):623-47. doi: 10.1016/s0950-3579(88)80032-3.
SLE is a syndrome defined by clinical criteria and by the presence of autoantibodies reactive with nucleic acids and proteins concerned with transcription and translation. Breeding experiments in mice have illustrated the enormous genetic heterogeneity of this syndrome, of which the final common pathway is a widespread immune complex disease. The causes of SLE in humans are likely to be equally multifactorial. Family studies have demonstrated that genetic factors exist, but each factor appears to be a relatively weak disease-susceptibility gene. The major exceptions to this are the very rare complete deficiencies of classical pathway complement components, which are almost invariably accompanied by the development of SLE. Observations of these patients have led to the formulation of hypotheses relating complement and its receptor, CR1, to the defective removal of immune complexes from the circulation.
系统性红斑狼疮（SLE）是一种由临床标准以及与核酸和参与转录及翻译的蛋白质发生反应的自身抗体的存在所定义的综合征。小鼠繁殖实验已表明该综合征存在巨大的遗传异质性，其最终共同途径是一种广泛的免疫复合物疾病。人类SLE的病因可能同样是多因素的。家族研究表明存在遗传因素，但每个因素似乎都是相对较弱的疾病易感性基因。主要的例外情况是非常罕见的经典途径补体成分完全缺乏，这几乎总是伴随着SLE的发展。对这些患者的观察导致了关于补体及其受体CR1与循环中免疫复合物清除缺陷相关的假说的形成。
