Atkinson J P
Howard Hughes Medical Institute Laboratories, Washington University School of Medicine, St. Louis, Missouri.
Am J Med. 1988 Dec 23;85(6A):45-7. doi: 10.1016/0002-9343(88)90383-x.
The recognition of the association between complete and partial complement (C) deficiencies and immune complex-mediated diseases is of clinical and etiopathologic interest. From studies of sera deficient in C1, C4, C2, or C3, the crucial role of these complement components in promoting the solubility and clearance of immune complexes has been elucidated. Moreover, partial C4 deficiency appears to be a common risk factor for the development of systemic lupus erythematosus, with complete C4A deficiency (C4A null) being present in 10 to 15 percent and heterozygous C4A deficiency present in 50 to 80 percent of patients with systemic lupus erythematosus. Most importantly, there is an obvious pathophysiologic relationship between the function of complement relative to immune complex processing and the disease that results from their deficiency. Systemic lupus erythematosus is characterized by excessive quantities of inappropriately deposited immune complexes. More subtle complement component and receptor deficiencies are likely to be predisposing factors for autoimmune disease. The complement deficiencies provide us with a unique opportunity to investigate the origin and development of immune complex excess syndromes.
认识到完全和部分补体（C）缺陷与免疫复合物介导的疾病之间的关联具有临床和病因病理学意义。通过对缺乏C1、C4、C2或C3的血清研究，已阐明这些补体成分在促进免疫复合物的溶解性和清除方面的关键作用。此外，部分C4缺陷似乎是系统性红斑狼疮发病的常见危险因素，在10%至15%的系统性红斑狼疮患者中存在完全C4A缺陷（C4A缺失），在50%至80%的系统性红斑狼疮患者中存在杂合性C4A缺陷。最重要的是，补体相对于免疫复合物处理的功能与因补体缺陷导致的疾病之间存在明显的病理生理关系。系统性红斑狼疮的特征是存在过量且沉积不当的免疫复合物。更细微的补体成分和受体缺陷可能是自身免疫性疾病的易感因素。补体缺陷为我们研究免疫复合物过多综合征的起源和发展提供了独特的机会。
