Goodpasture Syndrome Clinical Presentation

  • Author: Pranay Kathuria, MD, MBBS, FACP, FASN, FNKH; Chief Editor: Vecihi Batuman, MD, FACP, FASN   more...
 
Updated: Nov 16, 2011
 

History

Substantial variation exists in the clinical manifestations of patients with anti–glomerular basement membrane (anti-GBM) disease. From 60-80% of patients have clinically apparent manifestations of pulmonary and renal disease, 20-40% have renal disease alone, and less than 10% have disease that is limited to the lungs.

Symptoms include the following:

  • Constitutional symptoms like malaise, chills and fever, and/or arthralgias may precede or be concurrent with pulmonary or renal manifestations
  • Hemoptysis is the presenting symptom when the disease affects the lungs. The level of hemoptysis may vary and, in a small percentage of patients, may be absent. Other pulmonary symptoms include cough, dyspnea, and shortness of breath
  • Massive pulmonary hemorrhage leading to respiratory failure may occur
  • Chest pain is present in less than half of the patients
  • Renal manifestations include hematuria, edema, high blood pressure and eventually uremia
  • Significant anemia may result from persistent intrapulmonary bleeding
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Physical Examination

Physical examination findings in patients with anti-GBM disease include the following:

  • Tachypnea
  • Inspiratory crackles over lung bases
  • Cyanosis
  • Hepatosplenomegaly (may be present)
  • Hypertension (present in 20% of cases)
  • Rash
  • Edema
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Contributor Information and Disclosures
Author

Pranay Kathuria, MD, MBBS, FACP, FASN, FNKH  Professor of Medicine, Director, Division of Nephrology and Hypertension, University of Oklahoma School of Community Medicine

Pranay Kathuria, MD, MBBS, FACP, FASN, FNKH is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Heart Association, American Society of Hypertension, American Society of Nephrology, and National Kidney Foundation

Disclosure: Nothing to disclose.

Coauthor(s)

Frazier T Stevenson, MD  Associate Professor of Clinical Medicine and Director of Education Development, University of California Davis School of Medicine

Disclosure: Nothing to disclose.

Sat Sharma, MD, FRCPC  Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St Boniface General Hospital

Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, and World Medical Association

Disclosure: Nothing to disclose.

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.

Additional Contributors

Eleanor Lederer, MD Professor of Medicine, Chief, Nephrology Division, Director, Nephrology Training Program, Director, Metabolic Stone Clinic, Kidney Disease Program, University of Louisville School of Medicine; Consulting Staff, Louisville Veterans Affairs Hospital

Eleanor Lederer, MD is a member of the following medical societies: American Association for the Advancement of Science, American Federation for Medical Research, American Society for Biochemistry and Molecular Biology, American Society for Bone and Mineral Research, American Society of Nephrology, American Society of Transplantation, International Society of Nephrology, Kentucky Medical Association, National Kidney Foundation, and Phi Beta Kappa

Disclosure: Dept of Veterans Affairs Grant/research funds Research

James W Lohr, MD Professor, Department of Internal Medicine, Division of Nephrology, Fellowship Program Director, University of Buffalo State University of New York School of Medicine and Biomedical Sciences

James W Lohr, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Nephrology, and Central Society for Clinical Research

Disclosure: Genzyme Honoraria Speaking and teaching

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

Mauro Verrelli, MD, FRCP(C), FACP Assistant Professor, Department of Medicine, Section of Nephrology, University of Manitoba, Canada

Mauro Verrelli, MD, FRCP(C), FACP is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Society of Nephrology, Canadian Medical Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

References

  1. Zhao J, Cui Z, Yang R, Jia XY, Zhang Y, Zhao MH. Anti-glomerular basement membrane autoantibodies against different target antigens are associated with disease severity. Kidney Int. Nov 2009;76(10):1108-15. [Medline].

  2. Donaghy M, Rees AJ. Cigarette smoking and lung haemorrhage in glomerulonephritis caused by autoantibodies to glomerular basement membrane. Lancet. Dec 17 1983;2(8364):1390-3. [Medline].

  3. Yang R, Cui Z, Zhao J, Zhao MH. The role of HLA-DRB1 alleles on susceptibility of Chinese patients with anti-GBM disease. Clin Immunol. Nov 2009;133(2):245-50. [Medline].

  4. Savage CO, Pusey CD, Bowman C, Rees AJ, Lockwood CM. Antiglomerular basement membrane antibody mediated disease in the British Isles 1980-4. Br Med J (Clin Res Ed). Feb 1 1986;292(6516):301-4. [Medline]. [Full Text].

  5. Shah MK, Hugghins SY. Characteristics and outcomes of patients with Goodpasture's syndrome. South Med J. Dec 2002;95(12):1411-8. [Medline].

  6. Weber MF, Andrassy K, Pullig O, Koderisch J, Netzer K. Antineutrophil-cytoplasmic antibodies and antiglomerular basement membrane antibodies in Goodpasture's syndrome and in Wegener's granulomatosis. J Am Soc Nephrol. Jan 1992;2(7):1227-34. [Medline].

  7. Collard HR, Schwarz MI. Diffuse alveolar hemorrhage. Clin Chest Med. Sep 2004;25(3):583-92, vii. [Medline].

  8. Zhao J, Yan Y, Cui Z, Yang R, Zhao MH. The immunoglobulin G subclass distribution of anti-GBM autoantibodies against rHalpha3(IV)NC1 is associated with disease severity. Hum Immunol. Jun 2009;70(6):425-9. [Medline].

  9. Sinico RA, Radice A, Corace C, Sabadini E, Bollini B. Anti-glomerular basement membrane antibodies in the diagnosis of Goodpasture syndrome: a comparison of different assays. Nephrol Dial Transplant. Feb 2006;21(2):397-401. [Medline].

  10. Yang R, Hellmark T, Zhao J, Cui Z, Segelmark M, Zhao MH, et al. Levels of epitope-specific autoantibodies correlate with renal damage in anti-GBM disease. Nephrol Dial Transplant. Jun 2009;24(6):1838-44. [Medline].

  11. Rutgers A, Slot M, van Paassen P. Coexistence of anti-glomerular basement membrane antibodies and myeloperoxidase-ANCAs in crescentic glomerulonephritis. Am J Kidney Dis. Aug 2005;46(2):253-62.

  12. Frankel SK, Cosgrove GP, Fischer A. Update in the diagnosis and management of pulmonary vasculitis. Chest. Feb 2006;129(2):452-65.

 
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Goodpasture syndrome. A 45-year-old man was admitted to the intensive care unit with respiratory failure secondary to massive hemoptysis and acute renal failure. The antiglomerular basement membrane antibodies were strongly positive. The autopsy showed consolidated lung from extensive bleeding, which led to asphyxiation.
Goodpasture syndrome. Close-up view of gross pathology in a 45-year-old man admitted to the intensive care unit with respiratory failure secondary to massive hemoptysis and acute renal failure. The antiglomerular basement membrane antibodies were strongly positive. The autopsy showed consolidated lung from extensive bleeding, which led to asphyxiation.
Cytoplasmic antineutrophilic cytoplasmic antibodies (c-ANCA), which can appear in Goodpasture syndrome, are also commonly observed in Wegener granulomatosis and other vasculitides.
Perinuclear antineutrophilic cytoplasmic antibodies (p-ANCA), which can appear in Goodpasture syndrome, are also observed in Churg-Strauss vasculitis and occasionally in Wegener granulomatosis.
This is a renal biopsy slide of a patient who presented with hemoptysis and hematuria. The renal biopsy revealed crescentic glomerulonephritis, which may be caused by systemic lupus erythematosus, vasculitis, or Goodpasture syndrome.
This image of direct immunofluorescence shows smooth linear staining of the basement membrane secondary to immunoglobulin G deposition. This confirms the diagnosis of Goodpasture syndrome. Image courtesy of K. Orr, MD.
Goodpasture syndrome. A 35-year-old man who previously smoked cigarettes heavily, developed massive hemoptysis. The blood work showed positive anti–glomerular basement membrane antibodies.
 
 
 
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