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Antiglomerular Basement Membrane Disease Follow-up

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

Further Outpatient Care

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  • 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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Further Inpatient Care

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  • 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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Inpatient & Outpatient Medications

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  • 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

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  • 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

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  • 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

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  • 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

See the list below:

  • 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, see eMedicineHealth'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 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.

References
  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].

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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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