How is Goodpasture syndrome (anti–glomerular basement membrane disease) (anti-GBM) treated?

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

The three principles of therapy in anti–glomerular basement membrane (anti-GBM) disease are as follows:

  1. Rapidly remove circulating antibody, primarily by plasmapheresis

  2. Stop further production of antibodies using immunosuppression with medications

  3. Remove offending agents that may have initiated the antibody production

The rapid institution of appropriate therapy depends on distinguishing anti-GBM disease from other pulmonary renal syndromes with similar presentations. Beginning therapy despite a pending or preliminary negative test result for serum anti-GBM antibodies may be necessary; a delay in this setting can be associated with adverse clinical outcomes.

Patients who develop massive hemoptysis or acute respiratory failure should be cared for in an intensive care unit (ICU). Transfer to a hospital where plasmapheresis and/or hemodialysis is available may be necessary. Standard indications for dialysis are followed.

After hospital discharge, patients require long-term regular visits for monitoring of renal function and immunosuppressive therapy. If renal function does not return, dialysis is continued indefinitely and the patient should be referred for renal transplantation.

Patients receiving renal transplants must be informed that anti-GBM disease can recur in the transplanted kidney, although graft loss due to this is very rare.

Go to Pediatric Anti-GBM Disease (Goodpasture Syndrome) for complete information on this topic.


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