From the Guidelines
Goodpasture syndrome should be treated with aggressive immunosuppression, including cyclophosphamide and glucocorticoids, plus plasmapheresis, as soon as the diagnosis is suspected, to rapidly remove circulating antibodies and suppress further antibody production, as recommended by the 2021 KDIGO clinical practice guideline for the management of glomerular diseases 1.
Treatment Overview
The treatment regimen typically involves:
- Corticosteroids, such as methylprednisolone 1g IV daily for 3 days, followed by oral prednisone 1mg/kg/day
- Cyclophosphamide, 2mg/kg/day orally
- Plasmapheresis, typically 4L exchanges daily or every other day for 2-3 weeks This approach aims to minimize morbidity, mortality, and improve quality of life by preventing permanent kidney damage and respiratory failure.
Key Considerations
- Early diagnosis and treatment are crucial, as delays can lead to permanent kidney damage or respiratory failure 1
- A kidney biopsy is helpful not only to confirm the diagnosis but also to provide valuable prognostic information that can guide the need for continued therapy 1
- Monitoring of anti-GBM antibody levels, kidney function, and respiratory status is essential during treatment
- Maintenance immunosuppression is typically continued for 6-12 months with gradual tapering of medications based on clinical response and antibody levels
Supportive Care
Patients often require supportive care, including:
- Dialysis for kidney failure
- Respiratory support for pulmonary hemorrhage These measures are critical to managing the complications of Goodpasture syndrome and improving patient outcomes.
From the Research
Definition and Characteristics of Goodpasture Syndrome
- Goodpasture syndrome is a rare autoimmune disorder characterized by pulmonary hemorrhage and glomerulonephritis 2.
- It typically affects young men and is rapidly progressive and fatal unless treated early 2.
- The syndrome is caused by antibodies directed against the noncollagenous domain of the alpha 3 chain of type IV collagen 2.
Treatment and Management
- Current combination therapy with plasmapheresis and immunosuppressive drugs is unlikely to be successful unless instituted early in appropriate patients 2.
- Plasmapheresis has been shown to be effective in removing antibodies and improving renal function in some patients 3, 4, 5.
- Immunosuppressive drugs, such as cyclophosphamide and prednisone, are also used to suppress antibody production and control the disease 3, 4, 5.
- Early diagnosis and prompt initiation of therapy can decrease disease progression and increase chances of survival 6.
Prognosis and Outcomes
- Before plasmapheresis was available, prognosis was bleak, and most patients died or were left with permanent renal impairment 2.
- With current treatment modalities, some patients can experience improvement in renal function and reduction in antibody levels 3, 4, 5.
- However, the disease can still be fatal if left untreated or if treatment is delayed 2, 6.