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About

Anti–glomerular basement membrane (anti-GBM) antibody disease is a rare autoimmune disorder in which circulating antibodies are directed against an antigen normally present in the GBM and alveolar basement membrane. The resultant clinical syndrome encompasses a spectrum ranging from mild or no renal involvement to rapidly progressive glomerulonephritis. Many patients develop pulmonary hemorrhage, and most individuals have signs of a generalized systemic illness.

Occurrence

Glomerulonephritis due to Anti-GBM antibody disease is rare. It occurs in less than 1 case per million persons.

Treatment & Prognosis

Treatment of anti-GBM disease is focused on removing the anti-GBM antibody from the blood. Although disease eradication is likely, it commonly results in permanent kidney damage, and the eventual need for a transplant. Before receiving a kidney transplant, it is recommended that patients wait at least 6 months after finishing treatment for anti-GBM disease. Once it is sure that the disease is no longer active, transplant is very safe. The chance that the anti-GBM disease will come back in the kidney occurs in less than 5% of patients.
Treatments include:

• Plasmapheresis
• Immunosuppression (via steroids, cyclophosphamide)
• Dialysis (if kidney function is compromised)