What is the prognosis of Goodpasture syndrome (anti–glomerular basement membrane disease) (anti-GBM)?

Updated: Dec 16, 2020
  • Author: Pranay Kathuria, MD, FACP, FASN, FNKF; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Answer

In the past, Goodpasture syndrome was usually fatal. Aggressive therapy with plasmapheresis, corticosteroids, and immunosuppressive agents has dramatically improved prognosis. [19] With this approach, the 5-year survival rate exceeds 80% and fewer than 30% of patients require long-term dialysis. In a study of patients patients admitted to intensive care units for acute manifestation of small-vessel vasculitis, including anti-GBM disease, delayed administration of cyclophosphamide was associated with a higher mortality rate. [20]

Patients presenting with serum creatinine levels greater than 4 mg/dL, oliguria, and more than 50% crescents on renal biopsy rarely recover. They usually progress to end-stage renal failure that requires long-term dialysis. In a retrospective analysis of patients with anti-GBM disease who started renal replacement therapy for end-stage renal disease (ESRD) in Australia and New Zealand (ANZDATA Registry), the median survival rate was 5.93 years with death predicted by older age and history of pulmonary hemorrhage. [18]


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