What is the role of immunosuppressive therapy 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

Immunosuppressive therapy is required to inhibit antibody production and rebound hypersynthesis, which may occur following discontinuation of plasma exchange. [33, 34, 37]

Initial therapy includes cyclophosphamide at 2 mg/kg orally, adjusted to maintain a white blood cell count of approximately 5000, and corticosteroids (eg, prednisone at 1-1.5 mg/kg). Treatment of acute life-threatening alveolar hemorrhage in patients with Goodpasture syndrome is with pulse methylprednisolone at 1 g/day for 3 days, followed by a gradual corticosteroid taper. Intravenous cyclophosphamide is begun concomitantly at 1 g/m2 and repeated 3-4 weeks later, depending on the recovery of bone marrow.

The duration of immunosuppressive therapy is not well established. Anti-GBM antibody levels must be monitored at regular intervals. In patients who achieve a prompt remission, immunosuppression with cyclophosphamide is continued for 2-3 months and steroids for 6 months. Patients with clinically or serologically active disease at 3-4 months need longer immunosuppression (6-9 mo). Azathioprine may be substituted for cyclophosphamide to reduce adverse effects, especially in patients needing prolonged immunosuppression.

Rituximab, a chimeric monoclonal antibody, effectively depletes CD20-positive B cells over 6-9 months and has been used in several case reports as an alternative approach in the treatment of anti-GBM antibody disease. In these reports, rituximab was used as either an initial or a second-line agent in patients in whom cyclophosphamide failed or yielded adverse effects. The anti-GBM antibodies became undetectable in all these patients, but they had variable renal outcomes. [38, 39]

Pneumocystis jiroveci pneumonia has an annual incidence of 1% but is a potentially deadly complication of immunosuppressive therapy in patients with Goodpasture syndrome. Prophylaxis with trimethoprim-sulfamethoxazole (160 mg trimethoprim and 800 mg sulfamethoxazole 3 times per week) may be a cost-effective method of prolonging life in these patients.


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