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    Drugs & Diseases > Pediatrics: General Medicine

    Anti-GBM Antibody Disease Treatment & Management

    Updated: Dec 19, 2019
    • Author: Agnieszka Swiatecka-Urban, MD, FASN, FAAP; Chief Editor: Craig B Langman, MD  more...
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    Treatment

    Medical Care

    Hospitalization is required for prompt diagnosis and treatment, close monitoring, and supportive care in patients with anti-glomerular basement membrane (GBM) antibody disease. Patients may initially require intensive care.

    • The therapeutic regimen depends on the patient's potential to respond.

      • Patients with moderate glomerulonephritis (serum creatinine level < 5 mg/dL and crescents in < 50-75% of glomeruli) and patients with acute disease (brief illness, lack of chronicity on histology) are likely to respond to therapy. The treatment of choice consists of repeated plasmapheresis combined with glucocorticosteroids and cyclophosphamide.

      • Patients with advanced disease (serum creatinine level >5 mg/dL and crescents in >75% of glomeruli) and histologic signs of chronicity are unlikely to improve with any therapy and should be spared the clinically significant risks of aggressive treatment. Supportive care and eventual renal transplantation are recommended.

      • Patients who are antineutrophilic cytoplasmic antibody (ANCA) positive with clinical presentations consistent with vasculitis are likely to benefit from aggressive therapy independent of the severity of disease. [11]

      • Most patients with pulmonary hemorrhage respond rapidly to methylprednisolone pulses, plasma exchange, or plasmapheresis.

      • Patients with mild renal disease who do not have pulmonary hemorrhage may be successfully treated with prednisone alone.

    • In patients with renal insufficiency, treatment should be commensurate with the severity of disease and includes therapy for hypertension, fluid overload, and electrolyte and acid-base imbalances.

    • Early plasmapheresis removes circulating anti-GBM antibodies and other mediators of inflammation and has been advocated as the treatment of choice.

      • Plasmapheresis with immunosuppression is effective in the treatment of pulmonary hemorrhage and substantially improves renal function in patients with serum creatinine levels of less than 7 mg/dL or with crescents in less than 50% of the glomeruli.

      • Therapy usually consists of 14 treatments during 2-3 weeks.

      • Concomitant administration of cyclophosphamide and steroids is essential to prevent rebound antibody formation.

      • Additional plasmapheresis may be required if anti-GBM antibody titers remain elevated after the treatments.

      • Patients undergoing plasmapheresis who develop serious infections benefit from intravenous administration of immunoglobulins.

    • Rituximab, a chimeric monoclonal antibody targeting the pan B-cell marker CD20, has been used as an adjunctive or second-line therapy in resistant cases or whencyclophosphamide is contraindicated. [12, 13]  At present, there is insufficient evidence to recommend it as a first-line therapy for patients with anti-GBM antibody disease. 

    • Experimental and future treatment

      • Preliminary data suggest that removal of anti-GBM antibody by means of immunoadsorption may be beneficial in patients with Goodpasture disease. These results must be verified before immunoadsorption can be recommended.

        • A retrospective review of 10 anti-GBM patients treated with immunoadsorption reported a reduction in antibodies to negative levels in all patients by the first 9 immunoadsorption treatments and that renal survival was 40% at diagnosis, 70% after the end of immunoadsorption, and 63% after one year. [14]   

    • The effect of blocking CD28-B7, the costimulatory pathway for T-cell activation, was evaluated in a rat model of anti-GBM disease. The rationale for this attempt was the observation that T-cell–mediated mechanisms may play a direct role in the glomerular and alveolar injury that occurs in anti-GBM disease.

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    Surgical Care

    See the list below:

    • In patients with irreversible renal failure, renal transplantation is usually deferred for at least 1 year to decrease the risk of recurrence.

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    Consultations

    See the list below:

    • A nephrologist may be needed to manage glomerulonephritis and renal insufficiency.

    • A pulmonologist may be needed to manage pulmonary hemorrhage.

    • An intense care specialist may need to be consulted to treat critically ill patients.

    • A surgeon may need to be consulted to establish dialysis access and perform renal transplantation.

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    Diet

    Dietary modifications for patients with renal insufficiency include the following:

    • Adjustments in fluid intake based on urine output

    • Eating foods with low levels of sodium and phosphate

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    Activity

    See the list below:

    • Patients should avoid strenuous activity.

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