7- RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS

• Rapidly Progressive Glomerulonephritis (RPGN)


  Loss of renal function over a short period of time progressing to end-stage renal disease in most untreated patients within a period of weeks to months
• RPGN


  • Characterized morphologically by extensive crescent formation (crescentic glomerulonephritis)
  • May present as a sequel to severe acute nephritic syndrome (gross hematuria, oliguria, hypertension, edema), or may have an insidious onset (fatigue, edema)
  • Hematuria is an essential finding +/- proteinuria
  • Renal size is normal
  • Poor prognostic signs are oliguria (<400ml/day) and GFR<15ml/min
• An accurate and urgent diagnosis is essential in the patient presenting with clinical findings suggestive of RPGN in order to save the kidney
• Patients should undergo appropriate serologic assays and a kidney biopsy
• Early initiation of appropriate therapy is essential to minimize the degree of irreversible renal injury (reversible if treated within a few months window)
• Management of RPGN


  1. Steroids (IV pulse for 3 days then oral)

  2. Cyclophosphamide or azathioprine or mycophenolate
• Types of RPGN


  • Immune Complex Mediated
  • Antibody Mediated
  • Pauci-Immune (Vasculitis)
• Immune Complex Mediated RPGN


  • Granular IF pattern
  • Low Complement
  • Normal Complement
  • Post-Strept GN
  • SLE
  • Membranoproliferative
  • Cryoglobulinemia
  • IgA nephropathy [HSP]
• Antibody Mediated RPGN


  • Linear IF pattern
  • Normal Complement
  • Anti-GBM (Goodpasture syndrome)
• Pauci-Immune (Vasculitis) RPGN


  • Negative or little IF pattern
  • Normal Complement
  • C-ANCA positive
  • P-ANCA positive
  • Wegner's Granulomatosis
  • Churg Strauss Syndrome