Fibrillary glomerulonephritis with small fibrils in a patient with the antiphospholipid antibody syndrome successfully treated with immunosuppressive therapy

BMC Nephrol. 2007 May 9;8:7. doi: 10.1186/1471-2369-8-7.

Abstract

Background: Fibrillary glomerulonephritis is a rare cause of progressive renal dysfunction, often leading to the need for dialysis within a few years. The role of immunosuppressive treatment is still uncertain although this has been tried with variable success.

Case presentation: A 56 year old woman with the antiphospholipid antibody syndrome (IgM anticardiolipin antibodies) was seen in the nephrology clinic with haematuria, proteinuria, and worsening renal function. A renal biopsy demonstrated a mesangial proliferative glomerulonephritis on light microscopy and smaller fibrils (10.6-13.8 nm in diameter) than is usual for fibrillary glomerulonephritis (typically 18-22 nm) on electron microscopy. Amyloidosis was excluded following detailed evaluation. On account of rapidly worsening renal failure she was started on cyclophosphamide and prednisolone which led to the partial recovery and stabilization of her renal function.

Conclusion: This case highlights the need for routine electron microscopy in native renal biopsies, where the differential diagnosis is wide and varied and the light and immunofluorescence microscopic findings may be non specific.

Publication types

  • Case Reports

MeSH terms

  • Antiphospholipid Syndrome / diagnosis
  • Antiphospholipid Syndrome / drug therapy*
  • Cyclophosphamide / administration & dosage*
  • Drug Combinations
  • Female
  • Glomerulonephritis / diagnosis
  • Glomerulonephritis / drug therapy*
  • Humans
  • Immunosuppressive Agents / administration & dosage*
  • Middle Aged
  • Prednisolone / administration & dosage*
  • Treatment Outcome

Substances

  • Drug Combinations
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Prednisolone