Rynes R I, Urizar R E, Pickering R J
Clin Exp Immunol. 1978 Apr;32(1):59-68.
Two patients with systemic lupus erythematosus associated with homozygous deficiency of the second complement component (SLE-C2D) illustrate the different clinical disease patterns found in patients with this illness. Despite the differences in extent and severity of clinical manifestations and serological findings, the renal disease was similar and kidney function was well preserved in both patients. Renal microscopic changes were focal and segmental, deposits of immuno-globulins and complement components were present by immunofluorescent staining, and dense deposits were seen by electron microscopy. Tubulo-reticular inclusion bodies were found in glomerular endothelial cells and lymphocytes of both patients, but not in the lymphocytes of a clinically healthy C2D sibling. The findings in these two patients stress the importance of careful evaluation to determine the presence of systemic disease in patients with SLE-C2D and suggest that an intact classic complement pathway is important in the development of severe lupus, nephritis, but is not needed in the pathogenesis of lupus skin lesions.
两名患有系统性红斑狼疮合并第二补体成分纯合缺陷（SLE-C2D）的患者，展现了该疾病患者中发现的不同临床疾病模式。尽管临床表现和血清学检查结果在程度和严重程度上存在差异，但两名患者的肾脏疾病相似且肾功能均保存良好。肾脏显微镜检查变化为局灶性和节段性，免疫荧光染色显示有免疫球蛋白和补体成分沉积，电子显微镜下可见致密沉积物。在两名患者的肾小球内皮细胞和淋巴细胞中均发现了管网状包涵体，但在临床健康的C2D同胞的淋巴细胞中未发现。这两名患者的研究结果强调了仔细评估以确定SLE-C2D患者是否存在系统性疾病的重要性，并表明完整的经典补体途径在严重狼疮性肾炎的发展中很重要，但在狼疮皮肤病变的发病机制中并非必需。
