Husek K, Surel S, Zharfbin A
I. patologickoanatomický ústav, FN u sv. Anny, Brno.
Cesk Patol. 2002 Apr;38(2):75-82.
Fibrillary glomerulonephritis (FGN) is a rare cause of nephrotic syndrome. In three patients (65-year-old male; 57- and 38-year-old females) FGN was diagnosed by percutaneous renal biopsy. Clinically, the disease manifested itself with long term proteinuria and nephrotic syndrome without any associated systemic disorder. Histologically, glomeruli showed deposition of PAS+, fuchsinophilic and Orange G+ material in the mesangium and basal membranes. Strong granular immunofluorescence IgG, C3 and weak kappa and lambda light chains mesangial and peripheral deposition were observed in two patients. Electronmicroscopically, deposition of fibrillary material into mesangium and basal membranes was found. Randomly distributed nonbranching fibrils were 18-28 nm thick. After 10 to 13 months of follow-up, the therapy with corticosteroids and immunosuppressives was without effect, and the disease progressed into chronic renal failure in two patients; the third patient did well.
纤维性肾小球肾炎（FGN）是肾病综合征的一种罕见病因。在三名患者（一名65岁男性；两名分别为57岁和38岁的女性）中，经皮肾活检确诊为FGN。临床上，该疾病表现为长期蛋白尿和肾病综合征，无任何相关的全身性疾病。组织学上，肾小球在系膜和基底膜显示PAS+、嗜品红和橘黄G+物质沉积。在两名患者中观察到强颗粒状免疫荧光IgG、C3以及弱κ和λ轻链系膜和外周沉积。电子显微镜下，发现纤维状物质沉积于系膜和基底膜。随机分布的无分支纤维直径为18 - 28纳米。经过10至13个月的随访，使用皮质类固醇和免疫抑制剂治疗无效，两名患者病情进展为慢性肾衰竭；第三名患者情况良好。
