title: Systemic non-amyloidotic fibril deposition disease: a probable variant form of fibrillary glomerulonephritis.
abstract: 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.
authors.affiliation: Department of Nephrology, Iwate Prefectural Central Hospital, Morioka, Japan. sjun@chuo-hp.jp
authors.forename: J
authors.initials: J
authors.lastname: Soma
authors.forename: K
authors.initials: K
authors.lastname: Sato
authors.forename: I
authors.initials: I
authors.lastname: Nakaya
authors.forename: M
authors.initials: M
authors.lastname: Yahata
authors.forename: T
authors.initials: T
authors.lastname: Sakuma
authors.forename: H
authors.initials: H
authors.lastname: Sato
mesh_terms: D001344:Autopsy; D001706:Biopsy; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D005921:Glomerulonephritis / Q000150:complications / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007668:Kidney / Q000473:pathology*; D008100:Liver Abscess / Q000150:complications; D008875:Middle Aged; D011507:Proteinuria / Q000209:etiology; D006435:Renal Dialysis; D051437:Renal Insufficiency / Q000209:etiology / Q000628:therapy