Velosa J A, Donadio J V, Holley K E
Mayo Clin Proc. 1975 Mar;50(3):121-33.
Forty cases of focal sclerosing glomerulonephropathy with nephrotic syndrome or proteinuria were studied retrospectively in regard to clinical presentation, response to steroid therapy and clinical course, and histopathology of the lesion. Morphologically there was a focal segmental and global sclerosis with subendothelial hyaline deposits, collapse of the capillary loops, intracapillary hyaline material or foam cells, filling and widening of the mesangium with mesangial matrix, focal tubular atrophy, and focal interstitial fibrosis. Thirty-four patients had been treated with prednisone; initial complete remission of the nephrotic syndrome occurred in only 4 patients and partial remission in 10. Nine of these 14 patients had nephrotic relapse or became resistant to steroids. Thirty-three percent of the patients progressed to end-stage renal failure and an additional 25 percent had impairment of renal function after a mean of 8 years from onset. Three patients received kidney allografts, and in two the disease recurred in the transplanted kidney. Focal sclerosing glomerulonephropathy associated with nephrotic syndrome or proteinuria appears to be a clinicopathologic entity characterized by resistance to steroid treatment, frequent progression to end-stage renal disease, and recurrence in the transplanted kidney.
对40例患有肾病综合征或蛋白尿的局灶性硬化性肾小球肾炎患者进行了回顾性研究，内容包括临床表现、对类固醇治疗的反应及临床病程，以及病变的组织病理学。形态学上可见局灶节段性和全球性硬化，伴有内皮下透明样沉积物、毛细血管襻塌陷、毛细血管内透明样物质或泡沫细胞、系膜基质填充和增宽系膜、局灶性肾小管萎缩以及局灶性间质纤维化。34例患者接受了泼尼松治疗；仅4例患者肾病综合征最初完全缓解，10例部分缓解。这14例患者中有9例肾病复发或对类固醇耐药。33%的患者进展为终末期肾衰竭，另有25%的患者在发病平均8年后出现肾功能损害。3例患者接受了同种异体肾移植，其中2例移植肾疾病复发。与肾病综合征或蛋白尿相关的局灶性硬化性肾小球肾炎似乎是一种临床病理实体，其特征为对类固醇治疗耐药、频繁进展为终末期肾病以及移植肾复发。
