Adeyi O A, Sethi S, Rennke H G
Department of Pathology, Brigham and Women's Hospital, Boston, MA, USA.
Hum Pathol. 2001 Jun;32(6):660-3. doi: 10.1053/hupa.2001.25005.
Deposition of nonamyloid fibrillary material in glomeruli is well known. It is, however, unusual to find these fibrils in the tubular basement membranes and unprecedented to have fibrils of different sizes in the same patient. We present 2 cases with nephrotic range proteinuria with evidence of renal insufficiency. In both cases, strong, polyclonal immunoglobulin (Ig)G with C3 deposits were shown in the glomeruli and along tubular basement membranes. Ultrastructurally, the first case had 28-nm fibrils deposited extensively in the glomeruli and along tubular basement membranes. The second case had 30-nm fibrils in the glomeruli and 15-nm fibrils in the tubules. In both cases, the fibrils did not react with the regular amyloid stains. These findings are used to support the view that fibrillary glomerulopathy is not a disease, but rather the morphologic expression of an etiologically diverse group of diseases as yet incompletely defined.
肾小球中存在非淀粉样纤维状物质沉积是众所周知的。然而,在肾小管基底膜中发现这些纤维却并不常见,而在同一患者体内出现不同大小的纤维更是前所未有的。我们报告2例患有肾病范围蛋白尿且有肾功能不全证据的病例。在这2例病例中,肾小球及肾小管基底膜均显示有强阳性的多克隆免疫球蛋白(Ig)G伴C3沉积。超微结构检查显示,第1例有广泛沉积于肾小球及肾小管基底膜的28纳米纤维。第2例肾小球中有30纳米纤维,肾小管中有15纳米纤维。在这2例病例中,这些纤维均不与常规淀粉样染色反应。这些发现支持了以下观点,即纤维性肾小球病并非一种疾病,而是一组病因多样但尚未完全明确的疾病的形态学表现。