Antiglomerular Basement Membrane Disease Treatment & Management

Updated: May 23, 2018
  • Author: Ramesh Saxena, MD, PhD; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Medical Care

Antii–glomerular basement membrane (anti-GBM) nephritis is a rapidly progressive disease with a high mortality rate if not treated. Therefore, a prompt diagnosis and early treatment are of paramount importance in preventing death and preserving renal function.

The usual treatment for anti-GBM nephritis is with plasmapheresis in combination with intense immunosuppression consisting of corticosteroids and cyclophosphamide or azathioprine. Other therapeutic options include immunoadsorption using protein A affinity columns or treatment with cyclosporine. [15, 16, 17]


Since the first successful treatment by Lockwood and colleagues in 1976, plasmapheresis has become the standard treatment of anti-GBM nephritis. [18] This therapy effectively removes circulating anti-GBM antibodies and consists of removal of 1 volume of plasma (usually 4 L) and replacement with an equal volume of 5% albumin.

Plasmapheresis is continued daily until anti-GBM antibodies are undetectable in the blood. Usually, 10-14 treatments are required.

In patients with pulmonary hemorrhage, replace clotting factors by administering fresh frozen plasma at the end of treatment.

An early series of studies suggested that oliguric patients and those on dialysis before treatment rarely improve with plasmapheresis. However, more recent reports suggest that patients with advanced renal disease occasionally do respond, particularly if they are not anuric or if the biopsy specimen reveals a low proportion of sclerosed glomeruli.

Protein A immunoadsorption

Several investigators have reported the successful use of immunoadsorption with protein A in patients with anti-GBM nephritis who do not respond to plasmapheresis.

Protein A, isolated from the cell wall of the Staphylococcus aureus Cowan I strain, binds to the Fc portions of IgG. Thus, separated plasma from the patient is pumped through a protein A-Sepharose column to enable anti-GBM antibodies to bind, and the plasma is then returned to the patient. This prevents depletion of coagulation factors and other essential plasma proteins and obviates the need for large-volume replacement of fluids.


Immunosuppression usually includes high doses of steroids and cyclophosphamide. Cyclophosphamide is administered at a dose of 2-2.5 mg/kg/d. Adjust the dose of cyclophosphamide according to the degree of renal impairment. Administer cyclophosphamide for at least 1 year after remission, and then taper in 25-mg decrements every 2-3 months until discontinuation or disease recurrence.

Monitor the total leukocyte count frequently, and maintain it between 3000-5000/µL. High-dose steroids are also administered along with cyclophosphamide.

The role of pulse steroids is not clear in persons with anti-GBM nephritis, but some centers usually administer pulse steroids in patients with fulminant disease. Typically, methylprednisolone is given in dosages of 7-17 mg/kg/d for 3 consecutive days. Thereafter, oral prednisolone is started at a dosage of 1 mg/kg/d for 4 weeks and tapered slowly to 20 mg on alternate days by week 52 and withdrawn, as tolerated, thereafter (see Medication).



Consultation with pulmonary and critical care specialists is needed in patients presenting with hypoxia due to severe pulmonary hemorrhage. These patients may require intubation and mechanical ventilation. Furthermore, they may present with hemorrhagic shock and require hemodynamic monitoring in an intensive care unit.



Patients with renal failure should be placed on a diet with the following restrictions:

  • Sodium 2 g/day
  • Potassium  60 mEq/day
  • Protein 0.85 g/kg/day.