Kidney nontumor
Primary glomerular diseases
Fibrillary glomerulonephritis

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 15 September 2017, last major update April 2012

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Fibrillary glomerulonephritis [title] "loattrfree full text"[sb]

Cite this page: Sangle, N. Fibrillary glomerulonephritis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneyfibrillarygn.html. Accessed October 20th, 2017.
Definition / general
  • Not a disease but the morphologic expression of a diverse group of diseases incompletely defined (Hum Pathol 2001;32:660)
  • Deposition of extracellular nonamyloid fibrillary material in glomerular basement membranes, mesangium and tubular basement membranes ("nephropathy" better term than glomerulonephritis)
Clinical features
  • Rare, < 1% of renal biopsies
  • More common in whites and females
  • Associated with malignancy (most commonly carcinoma), dysproteinemia, autoimmune disease
  • Patients present with heavy proteinuria, hematuria, renal insufficiency, systemic hypertension
  • 50% develop end stage kidney disease within 2 - 4 years from diagnosis
  • No evidence of extrarenal fibrillary deposits
  • Features overlap with hepatitis C virus induced cryoglobulinemic glomerulonephritis
  • Diagnosis: based on ultrastructural (EM) findings; some authors require exclusion of cryoglobulins
Treatment
  • Kidney transplantation appears safe, with low risk of recurrence; in contast, patients with monoclonal gammopathy with fibrillary deposits have a significant risk for posttransplant recurrence (Kidney Int 2009;75:420)
  • Treatment with rituximab may be helpful (Am J Kidney Dis 2008;52:1158)
Case reports
Microscopic (histologic) description
  • Most common histologic patterns are mesangial proliferative / sclerosing followed by membranoproliferative glomerulonephritis (Clin J Am Soc Nephrol 2011;6:775)
  • Mesangial expansion with PAS positive material, diffuse thickening of the glomerular basement membrane
  • Variable proliferative lesions, 25% have crescents (Am J Kidney Dis 2012;60:157)
  • Silver stain highlights thickening of basement membrane and mesangial expansion by material that has a distinct "motheaten" appearance (due to silver negative deposits)
Microscopic (histologic) images

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Fibrillary glomerulonephritis

Case with crescents

Various images

IgG immunofluorescence

Immunofluorescence
  • Glomerular capillary wall and mesangial deposition of IgG4, C3, kappa and lambda light chains
Electron microscopy description
  • Randomly arranged nonamyloid fibrils in the mesangium and glomerular capillary walls, 18 - 22 nm thick vs. 10 nm thick fibrils for amyloid and 30 - 50 nm thick organized tubules for immunotactoid glomerulopathy
  • Usually extensive effacement of epithelial foot processes
Electron microscopy images

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Randomly oriented subepithelial fibrils

Fibrillar deposits in the mesangium