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Diagnosis: Anti-glomerular basement membrane disease

Anti-glomerular basement membrane (anti-GBM) is an autoimmune disease that clinically manifests as a rapidly progressive glomerulonephritis. When accompanied by pulmonary hemorrhage, it is designated the Goodpasture syndrome. It leads to acute renal failure and progression to end-stage renal disease in most cases. However, earlier diagnosis and better supportive care dramatically improved patient survival during the past decades. Plasmapheresis and immunosuppressive drugs are the most important treatment strategies. Two parameters have emerged as reliable predictors of adverse outcome: the fraction of crescentic glomeruli in biopsy specimens and anuria or a serum creatinine level greater than 5 mg/dL (442 µmol/L) at diagnosis. The autoantigen of anti-GBM disease is designated the Goodpasture antigen and comprises the noncollagenous domain (NC1) of the α-3 chain of type IV collagen. Linear IgG deposition along the GBM defines the disease (Fischer EG, Lager DJ. Anti-glomerular basement membrane glomerulonephritis: a morphologic study of 80 cases. Am J Clin Pathol. 2006;125:445-50. [PubMed link]).

Go to the chapter: Crescentic glomerulonephritis (anti-GBM and ANCA-associated glomerulonephritis) of our Tutorial

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References

• Tang W, McDonald SP, Hawley CM, Badve SV, Boudville NC, Brown FG, Clayton PA, Campbell SB, de Zoysa JR, Johnson DW. Anti-glomerular basement membrane antibody disease is an uncommon cause of end-stage renal disease. Kidney Int. 2013;83(3):503-10. [PubMed link]

  Lahmer T, Heemann U. Anti-glomerular basement membrane antibody disease: a rare autoimmune disorder affecting the kidney and the lung. Autoimmun Rev. 2012 Dec;12(2):169-73. [PubMed link]

  Cui Z, Zhao J, Jia XY, Zhu SN, Zhao MH. Clinical features and outcomes of anti-glomerular basement membrane disease in older patients. Am J Kidney Dis. 2011 Apr;57(4):575-82. [PubMed link]

  Kaplan AA. Anti-glomerular basement membrane disease in the elderly. Am J Kidney Dis. 2011;57(4):536-8. [PubMed link]

  Fischer EG, Lager DJ. Anti-glomerular basement membrane glomerulonephritis: a morphologic study of 80 cases. Am J Clin Pathol. 2006;125(3):445-50. [PubMed link]

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