What is the role of plasmapheresis 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

In published case series and one randomized trial, plasmapheresis has been shown to be beneficial in the treatment of Goodpasture syndrome by removal of anti-GBM antibodies. [33, 34, 35] Plasmapheresis is generally instituted after the diagnosis of Goodpasture syndrome is established either by renal biopsy or by detection of anti-GBM antibodies.

When a patient presents in a life-threatening situation secondary to pulmonary hemorrhage, however, plasmapheresis may be initiated if the diagnosis appears very likely, even though confirmation is not available immediately.

The extent and duration of plasmapheresis is not known, but 4-liter plasma exchanges daily or every other day is usually performed. The plasmapheresis is continued for 2-3 weeks or until the patient's clinical course has improved and serum anti-GBM antibodies are not detected.

A study by Zhang and colleagues in 28 patients with anti-GBM nephritis found that double filtration plasmapheresis cleared anti-GBM antibody about as effectively as immunoadsorption therapy (59.0% vs. 71.2% efficacy, respectively; P = 1.00). However, significantly fewer patients in the plasmapheresis group experienced reduced IgG (62.7% vs. 83.5%), and the plasmapheresis group also experienced fewer plasma-associated side effects. Patient survival and renal survival were similar in the two groups. [36]


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