Couser W G, Stilmant M M
Lab Invest. 1975 Nov;33(5):491-501.
The pathogenesis of focal glomerular sclerosis (FGS) and its relation to proteinuria and idiopathic nephrotic syndrome are unknown. Urine protein excretion in Sprague-Dawley rats increased with age. Fifty per cent of 12-month and 90 per cent of 24-month-old animals were proteinuric (greater than 20 mg. per day). Heavily proteinuric old rats manifested biochemical changes characteristic of nephrotic syndrome without significant loss of renal function. Three-month, 6-month, and nonproteinuric 12-month-old animals had mesangial deposits of IgM in occasional lobules of some glomeruli and slight mesangial hyperplasia. Four proteinuric 12-month-old rats had diffuse 4+ deposits of IgM in the mesangium of most glomeruli, basement membrane thickening and epithelial cell foot process fusion without FGS. The mesangial IgM deposits eluted in acid buffer and did not fix complement. Six proteinuric 12-month-old rats had focal and segmental areas of glomerular sclerosis with adhesions to Bowman's capsule, foamy cells, intraluminal eosinophilic deposits and capillary wall wrinkling and collapse. These lesions were more advanced in 24-month-old animals. Nonproteinuric 24-month-old rats did not have detectable FGS. Mesangial uptake of colloidal carbon was normal in proteinuric and nonproteinuric animals without FGS. Mesangial uptake of colloidal carbon was normal in proteinuric and nonproteinuric animals without FGS and reduced in proteinuric animals with FGS. In the aging rat the development of proteinuria and mesangial IgM deposition apparently precede development of a focal sclerotic glomerular lesion with histologic and ultrastructural features similar to FGS in man. The generalized impairment of mesangial phagocytic function in proteinuric rats with FGS suggests that this lesion may result from mesangial overload and dysfunction consequent to the persistent increase in glomerular permeability and proteinuria.
局灶性肾小球硬化(FGS)的发病机制及其与蛋白尿和特发性肾病综合征的关系尚不清楚。Sprague-Dawley大鼠的尿蛋白排泄量随年龄增加。12个月大的动物中有50%、24个月大的动物中有90%出现蛋白尿(每天超过20毫克)。重度蛋白尿的老龄大鼠表现出肾病综合征的生化变化,但肾功能无明显丧失。3个月、6个月和无蛋白尿的12个月大的动物在一些肾小球的偶尔小叶中有IgM系膜沉积和轻度系膜增生。4只蛋白尿的12个月大的大鼠在大多数肾小球的系膜中有弥漫性4+IgM沉积、基底膜增厚和上皮细胞足突融合,但无FGS。系膜IgM沉积在酸性缓冲液中洗脱,不固定补体。6只蛋白尿的12个月大的大鼠有局灶性和节段性肾小球硬化区域,伴有与鲍曼囊粘连、泡沫细胞、管腔内嗜酸性沉积以及毛细血管壁皱缩和塌陷。这些病变在24个月大的动物中更严重。无蛋白尿的24个月大的大鼠未检测到FGS。无FGS的蛋白尿和无蛋白尿动物的系膜对胶体碳的摄取正常。有FGS的蛋白尿动物的系膜对胶体碳的摄取减少。在老龄大鼠中,蛋白尿和系膜IgM沉积的发展显然先于具有与人FGS相似的组织学和超微结构特征的局灶性硬化性肾小球病变的发展。有FGS的蛋白尿大鼠系膜吞噬功能的普遍受损表明,这种病变可能是由于肾小球通透性和蛋白尿持续增加导致系膜超载和功能障碍所致。