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Antiglomerular Basement Membrane Disease Clinical Presentation

  • Author: Ramesh Saxena, MD, PhD; Chief Editor: Vecihi Batuman, MD, FACP, FASN  more...
Updated: Dec 04, 2015


Patients with anti-GBM nephritis can present with glomerulonephritis alone or with accompanying pulmonary hemorrhage.[8] Although pulmonary hemorrhage may be minor, it is often severe and life threatening. Pulmonary hemorrhage occurs more frequently in young adult males, whereas anti-GBM nephritis without lung involvement tends to occur more frequently in women in their seventh decade of life.[9]

The disease may begin with either renal or pulmonary manifestations. Usually, both organs are involved more or less simultaneously.[10] However, in several cases, the interval to the second organ's involvement may be prolonged up to a year.

  • Prodromal period
    • In 25-30% of patients, a prodromal period of flulike illness occurs.
    • In approximately 5% of patients, arthralgia, myalgia, and arthritis are prominent features.
  • Pulmonary manifestations
    • The onset of pulmonary hemorrhage may be insidious, with symptoms such as anemia, pallor, weakness, lethargy, dyspnea upon exertion, and, sometimes, dry cough.
    • In some cases, onset is acute and includes fever, massive hemoptysis, acute respiratory failure, asphyxia, and death; however, in many cases, the symptoms, including hemoptysis, dyspnea, cough, fever, tachycardia, and fatigue, may be present intermittently for weeks to months before the diagnosis is established.
  • Renal manifestations
    • The patient usually presents with an abrupt onset of oliguria or anuria. Hematuria or the passage of tea-colored urine is usually observed.
    • Rarely, the patient's renal involvement is more insidious in onset and he or she remains asymptomatic, progressing slowly until the development of uremic symptoms.


See the list below:

  • Physical examination in the acute stage of the disease reveals respiratory distress, tachycardia, and cyanosis.
  • The patient usually appears pale because of anemia.
  • In severe cases, the patient may be in hemorrhagic shock and in respiratory failure, thus requiring volume resuscitation and ventilatory support, respectively.
  • Chest examination may reveal fine rales and dullness to percussion over the affected lung areas.


The disease is caused by autoantibodies directed against the NC1 domain of the alpha-3 chain of type IV collagen.

  • Genetic susceptibility
    • Anti-GBM disease shows a strong association with HLA-DR2.
    • Further molecular genetics studies of HLA-DR2 reveal that the association of anti-GBM nephritis is with HLA-DRB1 alleles (HLA-DRB1 1501 and 1502 alleles), HLA-DQA1 01 alleles, and HLA-DQB1 06 alleles.
    • Anti-GBM nephritis is major histocompatibility complex–restricted. HLA-DRB1*1501 and 1502 alleles increase the susceptibility, while HLA-DR1 and HLA-DR7 are protective.
  • Environmental factors
    • A number of studies suggest a strong association between pulmonary hemorrhage and smoking.
    • Pulmonary hemorrhage may also be associated with exposure to hydrocarbons or other agents (eg, respiratory pathogens).
Contributor Information and Disclosures

Ramesh Saxena, MD, PhD 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: International Society for Peritoneal Dialysis, National Kidney Foundation, Texas Medical Association, American Society of Nephrology, International Society of Nephrology

Disclosure: Received honoraria from e-medicine for authoring review articles.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Christie P Thomas, MBBS, FRCP, FASN, FAHA Professor, Department of Internal Medicine, Division of Nephrology, Departments of Pediatrics and Obstetrics and Gynecology, 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 Heart Association, American Society of Nephrology, Royal College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Vecihi Batuman, MD, FACP, FASN Huberwald Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Renal Section, 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, International Society of Nephrology

Disclosure: Nothing to disclose.

Additional Contributors

Chike Magnus Nzerue, MD, FACP Professor of Medicine, Associate Dean for Clinical Affairs, Meharry Medical College

Chike Magnus Nzerue, MD, FACP 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, National Kidney Foundation

Disclosure: Nothing to disclose.

  1. Nagano C, Goto Y, Kasahara K, Kuroyanagi Y. Case report: anti-glomerular basement membrane antibody disease with normal renal function. BMC Nephrol. 2015 Nov 4. 16 (1):185. [Medline].

  2. Dammacco F, Battaglia S, Gesualdo L, Racanelli V. Goodpasture's disease: A report of ten cases and a review of the literature. Autoimmun Rev. 2013 Sep. 12(11):1101-8. [Medline].

  3. Hu SY, Jia XY, Yang XW, Yu F, Cui Z, Zhao MH. Glomerular C1q deposition and serum anti-C1q antibodies in anti-glomerular basement membrane disease. BMC Immunol. 2013 Sep 21. 14(1):42. [Medline].

  4. Sethi S, Haas M, Markowitz GS, D'Agati VD, et al. Mayo Clinic/Renal Pathology Society Consensus Report on Pathologic Classification, Diagnosis, and Reporting of GN. J Am Soc Nephrol. 2015 Nov 13. [Medline].

  5. Lerner RA, Glassock RJ, Dixon FJ. The role of anti-glomerular basement membrane antibody in the pathogenesis of human glomerulonephritis. J Exp Med. 1967 Dec 1. 126(6):989-1004. [Medline].

  6. Olson SW, Arbogast CB, Baker TP, Owshalimpur D, Oliver DK, Abbott KC, et al. Asymptomatic autoantibodies associate with future anti-glomerular basement membrane disease. J Am Soc Nephrol. 2011 Oct. 22(10):1946-52. [Medline].

  7. Xie LJ, Cui Z, Jia XY, Chen Z, Liu XR, Zhao MH. Coexistence of Anti-Glomerular Basement Membrane Antibodies and Anti-Neutrophil Cytoplasmic Antibodies in a Child With Human Leukocyte Antigen Susceptibility and Detailed Antibody Description: A Case Report. Medicine (Baltimore). 2015 Jul. 94 (29):e1179. [Medline].

  8. Sanders JS, Rutgers A, Stegeman CA, Kallenberg CG. Pulmonary: renal syndrome with a focus on anti-GBM disease. Semin Respir Crit Care Med. 2011 Jun. 32(3):328-34. [Medline].

  9. Kashif W, Yaqub S, Mahmood SF, Patel J. Double-positive Goodpasture's syndrome with concomitant active pulmonary tuberculosis. Saudi J Kidney Dis Transpl. 2013 Jul. 24(4):783-8. [Medline].

  10. Jia XY, Hu SY, Chen JL, Qu Z, Liu G, Cui Z, et al. The clinical and immunological features of patients with combined anti-glomerular basement membrane disease and membranous nephropathy. Kidney Int. 2013 Sep 18. [Medline].

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

  12. Cui Z, Zhao J, Jia XY, Zhu SN, Jin QZ, Cheng XY, et al. Anti-glomerular basement membrane disease: outcomes of different therapeutic regimens in a large single-center chinese cohort study. Medicine (Baltimore). 2011 Sep. 90(5):303-11. [Medline].

  13. Cui Z, Zhao MH. Advances in human antiglomerular basement membrane disease. Nat Rev Nephrol. 2011 Jul 19. [Medline].

  14. Mori M, Nwaogwugwu U, Akers GR, McGill RL. Anti-glomerular basement membrane disease treated with mycophenolate mofetil, corticosteroids, and plasmapheresis. Clin Nephrol. 2013 Jul. 80(1):67-71. [Medline].

  15. Lockwood CM, Rees AJ, Pearson TA, et al. Immunosuppression and plasma-exchange in the treatment of Goodpasture's syndrome. Lancet. 1976 Apr 3. 1(7962):711-5. [Medline].

  16. Wang XP, Fogo AB, Colon S, et al. Distinct epitopes for anti-glomerular basement membrane alport alloantibodies and goodpasture autoantibodies within the noncollagenous domain of alpha3(IV) collagen: a janus-faced antigen. J Am Soc Nephrol. 2005 Dec. 16(12):3563-71. [Medline].

  17. Kashtan CE. Renal transplantation in patients with Alport syndrome. Pediatr Transplant. 2006 Sep. 10(6):651-7. [Medline].

  18. Borza DB, Bondar O, Colon S, et al. Goodpasture autoantibodies unmask cryptic epitopes by selectively dissociating autoantigen complexes lacking structural reinforcement: novel mechanisms for immune privilege and autoimmune pathogenesis. J Biol Chem. 2005 Jul 22. 280(29):27147-54. [Medline].

  19. Borza DB. Autoepitopes and alloepitopes of type IV collagen: role in the molecular pathogenesis of anti-GBM antibody glomerulonephritis. Nephron Exp Nephrol. 2007. 106(2):e37-43. [Medline]. [Full Text].

  20. Butkowski RJ, Langeveld JP, Wieslander J, et al. Localization of the Goodpasture epitope to a novel chain of basement membrane collagen. J Biol Chem. 1987 Jun 5. 262(16):7874-7. [Medline].

  21. Bygren P, Freiburghaus C, Lindholm T, et al. Goodpasture's syndrome treated with staphylococcal protein A immunoadsorption. Lancet. 1985 Dec 7. 2(8467):1295-6. [Medline].

  22. Cameron JS. Recurrent renal disease after renal transplantation. Curr Opin Nephrol Hypertens. 1994 Nov. 3(6):602-7. [Medline].

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

  24. Goodpasture EW. The significance of certain pulmonary lesions in relation to the etiology of influenza. Am J Med Sci. 1919. 158:863-70.

  25. Gunnarsson A, Hellmark T, Wieslander J. Molecular properties of the Goodpasture epitope. J Biol Chem. 2000 Oct 6. 275(40):30844-8. [Medline].

  26. Hudson BG. The molecular basis of Goodpasture and Alport syndromes: beacons for the discovery of the collagen IV family. J Am Soc Nephrol. 2004 Oct. 15(10):2514-27. [Medline].

  27. Hudson BG, Tryggvason K, Sundaramoorthy M, et al. Alport's syndrome, Goodpasture's syndrome, and type IV collagen. N Engl J Med. 2003 Jun 19. 348(25):2543-56. [Medline].

  28. Hudson BG, Wieslander J, Wisdom BJ Jr, et al. Goodpasture syndrome: molecular architecture and function of basement membrane antigen. Lab Invest. 1989 Sep. 61(3):256-69. [Medline].

  29. Jung C, Karpouzas G, Stringer WW. Dyspnea, hemoptysis, and perihilar infiltrates in a 35-year-old man. Chest. 2005 Apr. 127(4):1437-41. [Medline].

  30. Khandelwal M, McCormick BB, Lajoie G, et al. Recurrence of anti-GBM disease 8 years after renal transplantation. Nephrol Dial Transplant. 2004 Feb. 19(2):491-4. [Medline].

  31. Larsson LA, Freiburghaus C, Nilsson IM. Plasma regeneration system for extensive immunoadsorption. Prog Artificial Organs. 1985. 902-4.

  32. Levy JB, Hammad T, Coulthart A, et al. Clinical features and outcome of patients with both ANCA and anti-GBM antibodies. Kidney Int. 2004 Oct. 66(4):1535-40. [Medline].

  33. Levy JB, Turner AN, Rees AJ, et al. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med. 2001 Jun 5. 134(11):1033-42. [Medline].

  34. Mathew TH. Recurrence of disease following renal transplantation. Am J Kidney Dis. 1988 Aug. 12(2):85-96. [Medline].

  35. Miner JH. Renal basement membrane components. Kidney Int. 1999 Dec. 56(6):2016-24. [Medline].

  36. Pirson Y, Goffin E, Squiffet JP. Outcome of anti-GBM nephritis after renal transplantation [abstract]. J Am Soc Nephrol. 1993. 4:955.

  37. Reynolds J, Moss J, Duda MA, et al. The evolution of crescentic nephritis and alveolar haemorrhage following induction of autoimmunity to glomerular basement membrane in an experimental model of Goodpasture's disease. J Pathol. 2003 May. 200(1):118-29. [Medline].

  38. Saus J, Wieslander J, Langeveld JP, et al. Identification of the Goodpasture antigen as the alpha 3(IV) chain of collagen IV. J Biol Chem. 1988 Sep 15. 263(26):13374-80. [Medline].

  39. Saxena R, Bygren P, Butkowski R, et al. Specificity of kidney-bound antibodies in Goodpasture's syndrome. Clin Exp Immunol. 1989 Oct. 78(1):31-6. [Medline].

  40. Saxena R, Isaksson B, Bygren P, et al. A rapid assay for circulating anti-glomerular basement membrane antibodies in Goodpasture syndrome. J Immunol Methods. 1989 Mar 10. 118(1):73-8. [Medline].

  41. Short AK, Esnault VL, Lockwood CM. ANCA and anti-GBM antibodies in RPGN. Adv Exp Med Biol. 1993. 336:441-4. [Medline].

  42. Simpson IJ, Doak PB, Williams LC, et al. Plasma exchange in Goodpasture's syndrome. Am J Nephrol. 1982. 2(6):301-11. [Medline].

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

  44. Stanton MC, Tange JD. Goodpasture's syndrome (pulmonary haemorrhage associated with glomerulonephritis). Australas Ann Med. 1958 May. 7(2):132-44. [Medline].

  45. Wang XP, Fogo AB, Colon S, et al. Distinct epitopes for anti-glomerular basement membrane alport alloantibodies and goodpasture autoantibodies within the noncollagenous domain of alpha3(IV) collagen: a janus-faced antigen. J Am Soc Nephrol. 2005 Dec. 16(12):3563-71. [Medline].

  46. Wieslander J, Bygren P, Heinegård D. Isolation of the specific glomerular basement membrane antigen involved in Goodpasture syndrome. Proc Natl Acad Sci U S A. 1984 Mar. 81(5):1544-8. [Medline].

  47. Wu J, Arends J, Borillo J, et al. A self T cell epitope induces autoantibody response: mechanism for production of antibodies to diverse glomerular basement membrane antigens. J Immunol. 2004 Apr 1. 172(7):4567-74. [Medline].

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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