Cheah P L, Looi L M, Ghazalli R, Chua C T
Department of Pathology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
Malays J Pathol. 1999 Jun;21(1):59-62.
A 31-year-old Malay female presented with nephrotic syndrome without renal impairment. Renal biopsy features were in keeping with immunotactoid glomerulopathy (ITG). Non-Congophilic deposits were seen causing thickening of the glomerular capillary basement membrane with segmental accentuation, and widening of the mesangium. Immunofluorescence examination showed moderate amounts of IgG and C3 in the glomerular capillary walls with some in the mesangium. Ultrastructurally, 20-nm thick fibrils with microtubular organisation were present predominantly in the subendothelial region with similar fibrils in the mesangium. Although immunotactoid glomerulopathy and fibrillary glomerulonephritis (FG) have been recognised as entities with extracellular fibrillary material in the kidney, to date much remains to be clarified regarding these 2 conditions. While the renal biopsy findings in this patient are consistent with ITG, her clinical presentation is unlike that of usual ITG in that she is of a much younger age and has no associated haemopoietic disorder. Response to initial treatment of 8 weeks of prednisolone therapy was poor.
一名31岁的马来女性患者出现肾病综合征但无肾功能损害。肾活检特征符合免疫触须样肾小球病（ITG）。可见非嗜刚果红沉积物，导致肾小球毛细血管基底膜增厚并呈节段性加重，系膜增宽。免疫荧光检查显示肾小球毛细血管壁有中等量的IgG和C3，系膜也有一些。超微结构上，20纳米厚的具有微管结构的纤维主要存在于内皮下区域，系膜中也有类似的纤维。虽然免疫触须样肾小球病和纤维样肾小球肾炎（FG）已被认为是肾脏中存在细胞外纤维物质的疾病，但迄今为止，关于这两种疾病仍有许多有待阐明的地方。虽然该患者的肾活检结果符合ITG，但她的临床表现与通常的ITG不同，因为她年龄小得多且无相关造血系统疾病。对泼尼松龙治疗8周的初始治疗反应不佳。
