eMedicine Specialties > Pediatrics: General Medicine > Nephrology

Anti-GBM Antibody Disease: Follow-up

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
Coauthor(s): Prasad Devarajan, MD, Louise M Williams Endowed Chair in Pediatrics, Professor of Pediatrics and Developmental Biology, Director of Nephrology and Hypertension, Director of Clinical Nephrology Laboratories, Chief Executive Officer of Dialysis Unit, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine
Contributor Information and Disclosures

Updated: Mar 31, 2009

Follow-up

Further Inpatient Care

  • 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.

Further Outpatient Care

  • 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.

Deterrence/Prevention

  • The patient should avoid exposure to known initiating factors, such as influenza, cigarette smoke,5 hydrocarbons, gasoline vapors, and hairsprays.

Complications

  • 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.

Prognosis

  • 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.

Patient Education

  • 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.

Miscellaneous

Medicolegal Pitfalls

  • Failure to consider the diagnosis in patients with hemoptysis and/or nephritis
  • Failure to consider the diagnosis in renal transplantation patients with deteriorating graft function and a previous history of Alport syndrome
  • Failure to recognize or inform patients of adverse effects of treatment
 


More on Anti-GBM Antibody Disease

Overview: Anti-GBM Antibody Disease
Differential Diagnoses & Workup: Anti-GBM Antibody Disease
Treatment & Medication: Anti-GBM Antibody Disease
Follow-up: Anti-GBM Antibody Disease
Multimedia: Anti-GBM Antibody Disease
References
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Further Reading

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Keywords

anti-glomerular basement membrane antibody disease, anti-GBM antibody disease, Goodpasture disease, Goodpasture's disease, Goodpasture syndrome, Goodpasture's syndrome, glomerulonephritis, pulmonary hemorrhage, renal failure, end-stage renal disease, nephritis, hematuria, oliguria, edema, treatment, diagnosis, tachycardia, tachypnea, hypertension, pulmonary rales, rash, skin rash, influenza, Alport syndrome

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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, FASN is a member of the following medical societies: American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, and Women in Nephrology
Disclosure: Nothing to disclose.

Coauthor(s)

Prasad Devarajan, MD, Louise M Williams Endowed Chair in Pediatrics, Professor of Pediatrics and Developmental Biology, Director of Nephrology and Hypertension, Director of Clinical Nephrology Laboratories, Chief Executive Officer of Dialysis Unit, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine
Prasad Devarajan, MD is a member of the following medical societies: American Heart Association, American Society of Nephrology, American Society of Pediatric Nephrology, National Kidney Foundation, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Uri S Alon, MD, Director of Research and Education, Department of Pediatrics, Division of Pediatric Nephrology, Children's Mercy Hospital of Kansas City; Professor, University of Missouri at Kansas City
Uri S Alon, MD is a member of the following medical societies: American Federation for Medical Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Adrian Spitzer, MD, Professor, Department of Pediatrics, Albert Einstein College of Medicine; Director of NIH Training Program, Children's Hospital at Montefiore Medical Center
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, and Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Howard Trachtman, MD, Program Director, Pediatrics Research, Schneider Children's Hospital, Department of Pediatrics, Division of Nephrology, Professor, Albert Einstein College of Medicine
Howard Trachtman, MD is a member of the following medical societies: American Society of Hypertension, American Society of Nephrology, American Society of Pediatric Nephrology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD, The Isaac A Abt, MD, Professor of Kidney Diseases, Feinberg School of Medicine, Northwestern University; Division Head of Kidney Diseases, Children's Memorial Hospital, Chicago
Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and International Society of Nephrology
Disclosure: Amgen Grant/research funds None; Altus Pharmaceuticals Grant/research funds None; Genzyme Grant/research funds None; Merck Grant/research funds None; NIH Grant/research funds None

 
 
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