7- RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS

Cards (10)

  • 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