Rapidly Progressive Glomerulonephritis

Features of acute nephritis, focal necrosis with/without crecents, and rapidly progressive renal failure over weeks; treatable cause of acute renal failure

Aetiology

  • ANCA positive → ANCA-associated Vasculitis
    • Systemic vasculitis - GPA, microscopic polyangiitis
  • ANCA negative
    • Goodpasture's disease - anti-GBM
    • HSP
    • SLE

Clinical presentation

  • Rapid deterioration in renal function over days/weeks
  • Active urinary sediment (RBCs, RBC and granular casts)
  • May be part of systemic disease

Investigations

  • Urine - active urinary sediment (RBCs, RBC and granular casts)
  • Renal biopsy - glomerular cresents
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Management

  • Immunosuppression
    • Steroids - IV methylprednisolone/oral prednisolone
    • Cytotoxics - cyclophosphamide/mycophenolate/azathioprine
  • Plasmapharesis
  • Supportive care including dialysis if needed