Goodpasture's syndrome

出自KMU Wiki

• Pulmonary-renal syndrome

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1. Goodpasture's syndrome
2. ANCA small-vessel vasculitis
3. Wegener's granulomatosis
4. Microscopic polyangitis
5. Churg-Strauss syndrome
6. Henoch-Schönlein purpura
7. Cryoglobulinemia

• D.D.
  • Lupus GN plus pneumonitis or pneumonia
  • Uremia plus pneumonia

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• Diagnostic triad
  • RPGN + Lung hemorrhage (hemoptysis) + anti-GBM antibody
  • Diagnosis
    • Serum anti-GBM antibody detected by antibody to α3 ~NC1 domain of type IV collagen
      • Serum anti-GBM antibody may be positive in patients with paraneoplastic syndrome if nonspecific anti-GBM antibodies are used
    • 10-15% p'ts also have serum ANCA to myeloperoxidase
      • Good prognosis
  • It appears in two age groups: in young men in late 20s and in men and women in 60–70s
  • Disease in the younger age group is usually explosive, with hemoptysis, a sudden fall in hemoglobin, fever, dyspnea, and hematuria. Hemoptysis is largely confined to smokers
  • Treatment
    • Plasmapheresis (for lung hemorrhage), steroids, cyclophosphamide
    • Renal transplantation (wait for 6 months and until serum antibodies are undetectable)
  • Prognosis
    • Poor if >50% crescents, advanced fibrosis, serum creatinine >5–6 mg/dL, oliguria, need for acute dialysis