What is the role of renal transplantation in the treatment 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

Renal transplantation has been used for end-stage renal disease secondary to Goodpasture syndrome. It is optimal to delay renal transplantation until anti-GBM antibodies are undetectable in the serum for 12 months and the disease has been in remission for at least 6 months without the use of cytotoxic agents.

Many patients develop linear deposits of IgG along glomeruli of the renal allograft. However, this development does not cause histologic or functional damage to the transplanted kidney.

Interestingly anti-GBM disease can occur in approximately 3-5% of male patients who have hereditary nephritis (Alport syndrome) undergoing renal transplantation, known as de novo anti-GBM disease. [40] In Alport syndrome (X-linked), the antibodies are directed against the alpha5 (IV) chain and, in patients who develop posttransplant anti-GBM disease, the antibody is directed against the alpha3 (IV) chain. Anti-GBM disease occurs most commonly in this subset of patients within the first year, and up to 75% patients have serum anti-GBM antibodies. The diagnosis is achieved with a renal biopsy with the characteristic findings of linear deposition of IgG along the capillaries and sometimes the distal tubules. Treatment consists of plasmapheresis and cyclophosphamide, but this appears to be limited. Retransplantation of patients with posttransplant anti-GBM nephritis has a very poor prognosis owing to recurrent disease.


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