Wegener's granulomatosis

• Facts:
  Pauci-immune glomerulonephritis ( nephritic syndrome )
  Systemic vasculitis, upper and lower airway granulomatous inflammation, and glomerulonephritis ("TRIAD")
• History / PE:
  Fever, weight loss
  Hemoptysis, hematuria
  Saddle nose deformity (nasal cartilage destruction)
  Hearing disturbances
  Cutaneous manifestations (palpable purpura, ulcerations)
  
• Diagnosis:
  Antineutrophilic cytoplasmic antibody (c-ANCA)
  Segmental necrotizing glomerulonephritis
  RBC casts, proteinura (UA)
• Treatment:
  High-dose corticosteroids
  Cytotoxic agents (eg. cyclophosphamide)
• See Also:
  Nephrotic syndrome
• Differential Diagnosis:
  Goodpasture's syndrome (anti-GBM)
  Churg-Strauss syndrome (p-ANCA)