What is the treatment for Goodpasture syndrome?

Treatment for Goodpasture Syndrome

The standard treatment for Goodpasture syndrome is immediate initiation of immunosuppression with cyclophosphamide and corticosteroids plus plasmapheresis in all patients except those who are dialysis-dependent with 100% crescents on biopsy and no pulmonary hemorrhage. 1, 2

Initial Management

Immediate Treatment

• Start treatment without delay once diagnosis is confirmed or highly suspected
• If diagnosis is highly suspected but not yet confirmed, begin treatment while awaiting confirmation 1

Standard Treatment Protocol

1. Plasmapheresis

   • Continue for approximately 14 days or until anti-GBM antibodies are undetectable 1
   • Typically requires 8-14 sessions to achieve undetectable antibody levels 2
   • Removes circulating anti-GBM antibodies rapidly 3, 4

2. Corticosteroids

   • Begin with IV pulse methylprednisolone
   • Follow with oral prednisone, tapering over at least 6 months 1, 2

3. Cyclophosphamide

   • Oral dosing at 2-3 mg/kg daily for approximately 3 months 1, 2
   • Dose adjustment required for reduced GFR and older age
   • Pneumocystis prophylaxis with trimethoprim-sulfamethoxazole required until cyclophosphamide is complete and prednisone dose is <20 mg daily 2

Special Considerations

Pulmonary Hemorrhage

• All patients with pulmonary hemorrhage should be treated regardless of renal status 1
• Hemoptysis often responds promptly to plasmapheresis 4
• Pulmonary involvement requires aggressive treatment due to life-threatening potential 5

Dialysis-Dependent Patients

• Patients who are dialysis-dependent at presentation with 100% crescents on biopsy and no pulmonary hemorrhage have poor renal prognosis (only ~8% recovery rate) 1
• Some clinicians advocate for a limited 4-8 week trial of treatment for functionally young patients with very rapid kidney function loss 1

ANCA Co-positivity

• Approximately 30% of anti-GBM patients are also ANCA positive ("double-positive") 2
• Double-positive patients should receive treatment as for anti-GBM disease initially 1
• These patients require maintenance immunosuppression as for ANCA-associated vasculitis 2

Seronegative Cases

• Some patients may have negative serum anti-GBM antibodies but positive linear IgG staining on kidney biopsy 6
• Kidney biopsy is crucial for diagnosis in seronegative cases 2

Maintenance Therapy

• No maintenance immunosuppressive therapy is recommended for isolated anti-GBM disease 1, 2
• Anti-GBM disease appears to be monophasic and self-limited, with antibodies often disappearing spontaneously after 6-18 months 1
• Relapse rate is <5% in properly treated patients 2
• Exception: Double-positive (ANCA + anti-GBM) patients require maintenance therapy as for ANCA-associated vasculitis 2

Monitoring and Follow-up

• Continue monitoring for at least 2 years after diagnosis 1
• Do not change immunosuppression based on ANCA titer changes alone 1
• Defer kidney transplantation until anti-GBM antibodies have been undetectable for a minimum of 6 months 1

Prognosis Factors

• Poor prognostic factors for renal recovery include:
  • Need for hemodialysis on initial presentation
  • Serum creatinine >5 mg/dL
  • 50-100% crescents on renal biopsy 5
• Early diagnosis and prompt treatment are critical for improved outcomes 6, 5

Common Pitfalls

• Delayed treatment leading to irreversible kidney damage
• Inadequate duration of plasma exchange resulting in persistent antibody presence
• Overtreatment of end-stage cases increasing complication risk
• Missing pulmonary involvement or overlooking double-positivity
• Failing to perform kidney biopsy in seronegative cases with clinical suspicion