Antiglomerular Basement Membrane Disease Treatment & Management

  • Author: Ramesh Saxena, MD, PhD; Chief Editor: Vecihi Batuman, MD, FACP, FASN   more...
 
Updated: Nov 21, 2011
 

Medical Care

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 uses 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.[5, 6]

  • Plasmapheresis
    • Since the first successful treatment by Lockwood and colleagues in 1976, plasmapheresis has become the standard treatment of anti-GBM nephritis.[7] The 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
    • 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).
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Consultations

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.

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Diet

Place patients with renal failure on a diet restricted to 2 g of sodium per day, 60 mEq of potassium per day, and 0.85 g/kg of protein per day.

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Activity

Patients may engage in activities as tolerated.

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Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Ramesh Saxena, MD, PhD  Associate Professor, Department of Internal Medicine, Division of Nephrology, University of Texas Southwestern Medical Center

Ramesh Saxena, MD, PhD is a member of the following medical societies: American Medical Association, American Society of Nephrology, International Society of Nephrology, and National Kidney Foundation

Disclosure: e-medicine Honoraria authoring review articles

Specialty Editor Board

Chike Magnus Nzerue, MD  Associate Dean for Clinical Affairs, Vice-Chairman of Internal Medicine, Meharry Medical College

Chike Magnus Nzerue, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Society of Nephrology, and National Kidney Foundation

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Christie P Thomas, MBBS, FRCP, FASN, FAHA  Professor, Department of Internal Medicine, Division of Nephrology, Medical Director, Kidney and Kidney/Pancreas Transplant Program, University of Iowa Hospitals and Clinics

Christie P Thomas, MBBS, FRCP, FASN, FAHA is a member of the following medical societies: American College of Physicians, American Federation for Medical Research, American Heart Association, American Society of Nephrology, American Society of Transplantation, American Thoracic Society, International Society of Nephrology, and Royal College of Physicians

Disclosure: Nothing to disclose.

Rebecca J Schmidt, DO, FACP, FASN  Professor of Medicine, Section Chief, Department of Medicine, Section of Nephrology, West Virginia University School of Medicine

Rebecca J Schmidt, DO, FACP, FASN is a member of the following medical societies: American College of Physicians, American Medical Association, American Society of Nephrology, International Society of Nephrology, National Kidney Foundation, Renal Physicians Association, and West Virginia State Medical Association

Disclosure: Renal Ventures Ownership interest Other

Chief Editor

Vecihi Batuman, MD, FACP, FASN  Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Medicine Service, Southeast Louisiana Veterans Health Care System

Vecihi Batuman, MD, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, and International Society of Nephrology

Disclosure: Nothing to disclose.

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Light microscopy of kidney biopsy specimen from a patient with antiglomerular basement membrane nephritis showing extensive crescent formation and the collapse of glomerular tuft.
Immunofluorescent examination of a kidney biopsy specimen from a patient with antiglomerular basement membrane nephritis showing a linear deposition of immunoglobulin G along the glomerular basement membrane.
 
 
 
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