MRCEM Notes

IgA nephropathy

Basics
  • also called Berger's disease or mesangioproliferative glomerulonephritis
  • commonest cause of glomerulonephritis worldwide
  • thought to be caused by mesangial deposition of IgA immune complexes
  • there is considerable pathological overlap with Henoch-Schonlein purpura (HSP)
  • histology: mesangial hypercellularity, positive immunofluorescence for IgA & C3

Presentations
  • young male, recurrent episodes of macroscopic haematuria
  • typically associated with mucosal infections e.g., URTI
  • nephrotic range proteinuria is rare
  • renal failure

Differentiating between IgA nephropathy and post-streptococcal glomerulonephritis
  • post-streptococcal glomerulonephritis is associated with low complement levels
  • main symptom in post-streptococcal glomerulonephritis is proteinuria (although haematuria can occur)
  • there is typically an interval between URTI and the onset of renal problems in post-streptococcal glomerulonephritis


Associated conditions
  • alcoholic cirrhosis
  • coeliac disease/dermatitis herpetiformis
  • Henoch-Schonlein purpura

Management
  • steroids/immunosuppressants not be shown to be useful

Prognosis
  • 25% of patients develop ESRF
  • markers of good prognosis: frank haematuria
  • markers of poor prognosis: male gender, proteinuria (especially > 2 g/day), hypertension, smoking, hyperlipidaemia, ACE genotype DD

© Image used on license from PathoPic
Proliferation and hypercellularity of the mesangium is seen in the glomerulus

© Image used on license from PathoPic
Immunostaining for IgA in a patient with HSP
Full-size image