Systemic non-amyloidotic fibril deposition disease: A probable variant form of fibrillary glomerulonephritis

J. Soma, K. Sato, I. Nakaya, M. Yahata, T. Sakuma, Hiroshi Sato

Research output: Contribution to journalArticlepeer-review

1 Citation (Scopus)


Fibrillary glomerulonephritis (FGN) is characterized by deposition of nonamyloidotic 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.

Original languageEnglish
Pages (from-to)74-79
Number of pages6
JournalClinical nephrology
Issue number1
Publication statusPublished - 2011 Jan 1


  • Amyloidosis
  • Fibrillary glomerulonephritis
  • Non-amyloidotic fibrils
  • Systemic deposition
  • Tubulointerstitial nephritis

ASJC Scopus subject areas

  • Nephrology


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