Goodpasture Syndrome Guidelines

Updated: Aug 23, 2023
  • Author: Pranay Kathuria, MD, MACP, FASN, FNKF; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Guidelines Summary

The KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases includes the following recommendations for the diagnosis and treatment of anti-GBM disease [42] :

  • In all patients with suspected rapidly progressive glomerulonephritis (RPGN), serologic testing for the presence of anti-GBM antibodies should be done urgently using commercially available enzyme-linked immunoassays.  In patients with negative results, kidney biopsy is required for diagnosis of anti-GBM disease.
  • Treatment for suspected anti-GBM should be initiated before the diagnosis is confirmed
  • Treat anti-GBM disease with cyclophosphamide and glucocorticoids plus plasmapheresis except those who are on dialysis at presentation, have 100% crescents or >50% global glomerulosclerosis in an adequate biopsy sample, and do not have pulmonary hemorrhage.
  • Plasma exchange should be performed until anti-GBM titers are no longer detectable.
  • Cyclophosphamide should be administered for 2–3 months and glucocorticoids for about 6 months 
  • No maintenance therapy of anti-GBM disease is necessary; patients who are anti-GBM- and ANCA-positive should be treated with maintenance therapy as for patients with ANCA-associated vasculitis (AAV) 
  • Consider rituximab for refractory anit-GBM disease
  • Kidney transplantation in patients with kidney failure due to anti-GBM disease should be postponed until anti-GBM antibodies remain undetectable for ≥6 months