Anti-GBM Antibody Disease Follow-up

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

Further Outpatient Care

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  • After discharge, a nephrologist should follow up with the patient to monitor drug therapy, potential adverse effects, and renal function.

  • When necessary, the nephrologist should direct renal replacement therapy.

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Further Inpatient Care

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  • Care for critically ill patients with anti-glomerular basement membrane (GBM) antibody disease (eg, those with pulmonary hemorrhage, severe hypertension, or renal failure) in the ICU.

  • Acute dialysis is indicated in patients with anuria, pulmonary edema, uncontrolled hypertension, and hyperkalemia.

  • If renal function remains poor, prepare the patient for long-term dialysis.

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Deterrence/Prevention

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  • The patient should avoid exposure to known initiating factors, such as influenza, cigarette smoke, [15] hydrocarbons, gasoline vapors, and hairsprays.

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Complications

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  • Complications of renal failure include hyperkalemia, pulmonary edema, hypertension, and seizures.

  • Complications of pulmonary hemorrhage include hemorrhagic shock and respiratory failure.

  • Complications of immunosuppressive medications include infection, avascular bone necrosis, and bone marrow suppression.

  • Complications of plasmapheresis include infection, bleeding, hypocalcemia, and immunoglobulin deficiency.

  • Complications of renal transplantation include a recurrence rate of linear immunoglobulin G (IgG) staining in the graft as high as 50%. However, most patients remain asymptomatic, probably because of inhibition of autoantibody production with routine posttransplantational immunosuppression. The risk of graft loss due to recurrent anti-GBM disease is low.

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Prognosis

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  • The prognosis is poor but not uniform. Without treatment, 90% of patients progress to dialysis or die, and only 10% improve. With current therapies, improvement occurs in 50%. Patients who survive the first year with normal renal function have a good long-term prognosis, though late relapses can occur. Several clinical, laboratory, and histologic features have prognostic relevance independent of the type of therapy.

  • Chronic disease (weeks vs days), a need for dialysis, a serum creatinine level of more than 5 mg/dL, and crescent formation in 50-75% of the glomeruli at the time of diagnosis are associated with a poor outcome. Other histologic findings, including fibrous crescents, widespread necrosis, and tubulointerstitial changes, indicate advanced disease and a high likelihood of progression to renal failure.

  • Anti-GBM disease is usually non-repalsing. Patients who are antineutrophilic cytoplasmic antibody (ANCA) positive and who have a clinical course resembling that of vasculitis tend to respond well to treatment and recover renal function despite an increased frequency of vasculitic relapses. Those patients may require maintenance immunosuppressive therapy to prevent relapses. [13]

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Patient Education

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  • Patients should seek prompt medical attention if symptoms of recurrent renal and/or pulmonary involvement, including cough, bloody sputum, oliguria, discoloration of urine, or edema, develop.

  • Patients should be informed about their long-term prognosis and the risks of treatment.

  • Patients should be made aware of known risk factors such as exposure to influenza, cigarette smoke, and inhaled toxins.

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