Soma J, Sato K, Nakaya I, Yahata M, Sakuma T, Sato H
Department of Nephrology, Iwate Prefectural Central Hospital, Morioka, Japan.
Clin Nephrol. 2011 Jan;75(1):74-9.
Fibrillary glomerulonephritis (FGN) is characterized by deposition of non-amyloidotic fibrillary material in glomeruli, and most patients with the disease show heavy proteinuria and hematuria, and progress into end-stage renal failure. We report a 62-year-old woman with FGN who showed mild proteinuria without hematuria and developed rapidly progressive renal failure requiring hemodialysis. Renal biopsy showed severe tubulointerstitial injury associated with non-amyloidotic fibrillary deposits in the tubular basement membrane, interstitium and vessel walls, in addition to glomeruli. The patient died from liver abscess 1 year after the introduction of hemodialysis. Postmortem examination showed the presence of non-amyloidotic fibrillary deposits arranged in tightly packed electron-dense and bundle-shaped structures in many organs. These findings suggest systemic non-amyloidotic fibril deposition in FGN.
纤维性肾小球肾炎（FGN）的特征是肾小球内有非淀粉样纤维物质沉积，大多数该疾病患者表现为大量蛋白尿和血尿，并进展为终末期肾衰竭。我们报告了一名62岁患有FGN的女性，她表现为轻度蛋白尿且无血尿，并迅速发展为需要血液透析的快速进行性肾衰竭。肾活检显示，除肾小球外，肾小管基底膜、间质和血管壁存在与非淀粉样纤维沉积物相关的严重肾小管间质损伤。该患者在开始血液透析1年后死于肝脓肿。尸检显示许多器官中存在排列紧密的电子致密束状结构的非淀粉样纤维沉积物。这些发现提示FGN存在系统性非淀粉样纤维沉积。
