Antiglomerular Basement Membrane Disease Follow-up

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

Further Inpatient Care

  • Patients presenting with respiratory failure may require intubation and mechanical ventilation. Furthermore, patients may present with hemorrhagic shock and require blood transfusion and hemodynamic monitoring. Patients presenting with advanced renal failure may require short- or long-term dialysis.
  • While in the hospital, patients should also receive supportive care (eg, adequate nutrition, prophylaxis for deep vein thrombosis and stress ulcers, good blood pressure control).
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Further Outpatient Care

  • After discharge from the hospital, patients need a detailed follow-up visit with a nephrologist. Renal function should be closely monitored for progression of renal disease or recurrence of anti-GBM nephritis. Patients should also have their blood cell counts checked frequently while they are taking cyclophosphamide because they are at high risk for hemorrhagic cystitis or transitional cell carcinoma of the urinary bladder. Therefore, their urine should be screened for nonglomerular hematuria; all patients with nonglomerular hematuria should undergo a further urological evaluation.
  • Patients with anti-GBM nephritis who are taking high doses of steroids are at a high risk of developing osteoporosis. Therefore, they should receive calcium and vitamin D supplements (1000 mg/d elemental calcium for men and 1500 mg/d for women). Bone densitometry may also be performed to check for osteoporosis.
  • Patients receiving immunosuppression in high doses are also at risk for opportunistic infections. They should be placed on a single-strength tablet of trimethoprim-sulfamethoxazole per day for prophylaxis of Pneumocystis carinii infection. In addition, patients should rinse their mouths several times a day to prevent oral thrush and, if necessary, be given nystatin swish and swallows.
  • Patients who take high doses of steroids are prone to develop diabetes and hypertension. Thus, they should be screened for diabetes and have their blood pressure checked frequently.
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Inpatient & Outpatient Medications

  • Methylprednisolone at 7-17 mg/kg/d intravenously for 3 days should be administered to patients with fulminant disease.
  • The starting dose of prednisolone is 1 mg/kg/d. In patients receiving intravenous methylprednisolone, oral prednisolone should be started on the fourth day. Administration should be continued after discharge in tapering doses for one year.
  • Cyclophosphamide should be administered orally at 1 mg/kg/d. This agent should be started once the diagnosis is confirmed and should be continued in an outpatient setting for a total of one year.
  • Calcium carbonate at 500 mg orally 2-3 times/d should be administered for osteoporosis prevention.
  • Double-strength trimethoprim-sulfamethoxazole (Bactrim) or equivalent should be administered at a dose of one tablet every other day for prophylaxis of P carinii infection.
  • Omeprazole at 20 mg or an equivalent proton pump inhibitor should be administered once or twice a day for prophylaxis of stress ulcers.
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Transfer

  • Anti-GBM nephritis is a rare disease with a fulminant course if left untreated. The patient should be transferred to a well-equipped tertiary care center for prompt diagnosis and treatment.
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Complications

  • Respiratory failure
    • Patients with severe pulmonary hemorrhage may present with profound hypoxia and respiratory failure. They usually require admission to an intensive care unit for intubation and ventilation support. Furthermore, patients may present with hemorrhagic shock and may require blood transfusion and hemodynamic monitoring.
    • Patients presenting with advanced renal failure may require short- or long-term dialysis.
  • Hemorrhagic shock
    • In addition to respiratory failure, patients with severe pulmonary hemorrhage may present with hemorrhagic shock. They may require blood transfusion and hemodynamic monitoring. They also may require transfusion of other blood products, such as fresh frozen plasma and platelets, to replenish clotting factors and to prevent further bleeding.
    • Because many of these patients are young and are potential candidates for kidney transplantation, every attempt should be made to use leukocyte-poor blood to prevent allosensitization.
  • Renal failure
    • Patients with anti-GBM nephritis usually present with rapidly progressive renal failure.
    • Patients with an advanced degree of renal failure may require short- or long-term dialysis.
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Prognosis

  • Anti-GBM disease is an aggressive disease with a rapidly progressive course. In the early years, the mortality rate was extremely high (90-95%). With the introduction of immunosuppression and plasmapheresis, the prognosis has improved considerably, with patient and renal survival rates of approximately 85% and 60%, respectively. Both renal survival and patient survival depend on the severity of the disease at the time of presentation. The following reported survival rates underscore the importance of rapid diagnosis and prompt institution of aggressive immunosuppression therapy for patients with Goodpasture syndrome and severe renal failure.
    • Patients who present with a serum creatinine level of less than 500 µmol/L (5.7 mg/dL) have 1-year patient and renal survival rates of 100% and 95%, respectively.
    • Patients who present with a serum creatinine level of more than 500 µmol/L (5.7 mg/dL) but do not require dialysis have 1-year patient and renal survival rates of 83% and 82%, respectively.
    • Patients who present with dialysis-dependent renal failure have 1-year patient and renal survival rates of 65% and 8%, respectively.
  • Importantly, patients with advanced renal disease at the time of presentation (ie, oliguric or dialysis dependent) do not usually respond to plasmapheresis, methylprednisolone, or other immunosuppressive therapy.
  • Other poor prognostic factors include extensive crescent formation (>50%), the presence of significant tubular atrophy, interstitial fibrosis or glomerulosclerosis, oliguria or anuria, and a serum creatinine level of more than 6 mg/dL. Patients with HLA-DR W2 and HLA-B7 appear to have a more malignant course.
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Patient Education

  • Risk of bladder cancer
    • Patients with anti-GBM nephritis receive large doses of cyclophosphamide for a prolonged period. This makes them high-risk candidates for the development of hemorrhagic cystitis and bladder cancer. They should drink large quantities of water to ensure urine output of at least 2 L/d. Patients should avoid becoming dehydrated.
    • They should also watch for gross hematuria and report it promptly to their physician. Patients should have regular urinalyses to screen for nonglomerular hematuria.
    • Furthermore, smoking has been shown to increase the risk of bladder cancer in patients receiving cyclophosphamide; therefore, patients should be encouraged to quit smoking.
  • Risk of osteoporosis
    • Patients are at a high risk for developing steroid-induced osteoporosis. They should be encouraged to take adequate calcium in their diets and to take additional calcium supplements.
    • Postmenopausal women should also receive estrogen.
  • Risk of opportunistic infections
    • Intense immunosuppression can make patients susceptible to opportunistic infections. Therefore, patients should be advised to avoid close contact with ill people.
    • They should receive prophylaxis against certain infections (eg, P carinii, yeast) and should contact their physician if they develop fever, sore throat, cough with expectoration, or any other signs of infection.
  • For excellent patient education resources, visit eMedicine's Kidneys and Urinary System Center. Also, see eMedicine's patient education article Blood in the Urine.
  • For further information, see Mayo Clinic - Kidney Transplant Information.
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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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