Miyazaki R, Kuroda M, Akiyama T, Otani I, Tofuku Y, Takeda R
Clin Nephrol. 1984 Jun;21(6):335-40.
We examined complement control proteins focusing on the role of modulating the complement activation in IgA nephropathy. Glomerular C4-binding protein (C4-bp) deposits were found in 60% of patients with IgA nephropathy, while C4 deposits were found in 30%. Glomerular deposits of beta 1H globulin (beta 1H) were found in 85% of patients with IgA nephropathy. The frequency of glomerular deposits of C4-bp tended to be higher in the group with deposits of various immunoglobulin types than in the group with deposits of IgA alone, and it increased parallel with the progression of glomerular histologic changes. The serum C4-bp level was higher in IgA nephropathy patients. No significant correlation was found between the serum level of C4-bp or beta 1H and the extent or distribution of tissue deposits of these complement control proteins. These results suggest that glomerular immune deposits in IgA nephropathy may be attributable to the activation not only of the alternative pathway but also of the classical pathway, the latter of which may take place locally in glomeruli with advanced histologic change or various immunoglobulin deposits in IgA nephropathy.
我们研究了补体调节蛋白，重点关注其在IgA肾病中调节补体激活的作用。在60%的IgA肾病患者中发现了肾小球C4结合蛋白（C4-bp）沉积，而C4沉积见于30%的患者。在85%的IgA肾病患者中发现了β1H球蛋白（β1H）的肾小球沉积。C4-bp肾小球沉积的频率在各种免疫球蛋白类型沉积的组中往往高于仅IgA沉积的组，并且它随着肾小球组织学变化的进展而增加。IgA肾病患者的血清C4-bp水平较高。未发现血清C4-bp或β1H水平与这些补体调节蛋白的组织沉积范围或分布之间存在显著相关性。这些结果表明，IgA肾病中的肾小球免疫沉积可能不仅归因于替代途径的激活，还归因于经典途径的激活，后者可能在IgA肾病中具有晚期组织学变化或各种免疫球蛋白沉积的肾小球局部发生。
