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Anti-GBM Antibody Disease Follow-up

  • Author: Agnieszka Swiatecka-Urban, MD; Chief Editor: Craig B Langman, MD  more...
 
Updated: May 28, 2014
 

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,[8] 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.
  • 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. Anti-GBM disease is usually not reactivated.
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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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Contributor Information and Disclosures
Author

Agnieszka Swiatecka-Urban, MD FASN, Assistant Professor, Department of Pediatrics, Cell Biology and Physiology, University of Pittsburgh School of Medicine; Assistant Professor, Department of Nephrology, Children's Hospital of Pittsburgh

Agnieszka Swiatecka-Urban, MD is a member of the following medical societies: American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, Women in Nephrology

Disclosure: Received consulting fee from Mallinckrodt Pharmaceuticals for consulting.

Coauthor(s)

Prasad Devarajan, MD, FAAP Louise M Williams Endowed Chair in Pediatrics, Professor of Pediatrics and Developmental Biology, Director of Nephrology and Hypertension, Director of the Nephrology Fellowship Program, Medical Director of the Kidney Stone Center, Co-Director of the Institutional Office of Pediatric Clinical Fellowships, Director of Clinical Nephrology Laboratory, CEO of Dialysis Unit, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine

Prasad Devarajan, MD, FAAP is a member of the following medical societies: American Heart Association, American Society of Nephrology, American Society of Pediatric Nephrology, National Kidney Foundation, Society for Pediatric Research

Disclosure: Received none from Coinventor on patents submitted for the use of NGAL as a biomarker of kidney injury for none.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Adrian Spitzer, MD Clinical Professor Emeritus, Department of Pediatrics, Albert Einstein College of Medicine

Adrian Spitzer, MD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Pediatric Society, American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD The Isaac A Abt, MD, Professor of Kidney Diseases, Northwestern University, The Feinberg School of Medicine; Division Head of Kidney Diseases, The Ann and Robert H Lurie Children's Hospital of Chicago

Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, International Society of Nephrology

Disclosure: Received income in an amount equal to or greater than $250 from: Alexion Pharmaceuticals; Raptor Pharmaceuticals; Eli Lilly and Company; Dicerna<br/>Received grant/research funds from NIH for none; Received grant/research funds from Raptor Pharmaceuticals, Inc for none; Received grant/research funds from Alexion Pharmaceuticals, Inc. for none; Received consulting fee from DiCerna Pharmaceutical Inc. for none.

Additional Contributors

Uri S Alon, MD Director of Bone and Mineral Disorders Clinic and Renal Research Laboratory, Children's Mercy Hospital of Kansas City; Professor, Department of Pediatrics, Division of Pediatric Nephrology, University of Missouri-Kansas City School of Medicine

Uri S Alon, MD is a member of the following medical societies: American Federation for Medical Research

Disclosure: Nothing to disclose.

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Immunofluorescence staining for immunoglobulin (IgG) reveals diffuse, high-intensity, linear staining of the glomerular basement membrane in a patient with anti–glomerular basement membrane (GBM) disease. Courtesy of Glen Markowitz, MD, Department of Pathology, Columbia University.
 
 
 
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