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Case 164
Diagnosis
 
     
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Diagnosis: Anti-Glomerular Basement Membrane Disease (Anti-GBM)

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]).

The frequency of pulmonary involvement is lower in older patients (Cui Z, et al. Clinical features and outcomes of anti-glomerular basement membrane disease in older patients. Am J Kidney Dis. 2011;57:575-82. [PubMed link]; Kaplan AA. Anti-glomerular basement membrane disease in the elderly. Am J Kidney Dis. 2011;57:536-8. [PubMed link])

 

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

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References

  • Marques C, Carvelli J, Biard L, Faguer S, Provôt F, Matignon M, Boffa JJ, Plaisier E, Hertig A, Touzot M, Moranne O, Belenfant X, Annane D, Quéméneur T, Cadranel J, Izzedine H, Bréchot N, Cacoub P, Piedrafita A, Jourde-Chiche N, Saadoun D. Prognostic Factors in Anti-glomerular Basement Membrane Disease: A Multicenter Study of 119 Patients. Front Immunol. 2019;10:1665. [PubMed link]
  • Segelmark M, Hellmark T. Anti-glomerular basement membrane disease: an update on subgroups, pathogenesis and therapies. Nephrol Dial Transplant. 2018 Oct 29. [Epub ahead of print] [PubMed link]
  • Liang D, Liang S, Xu F, Zhang M, Li X, Tu Y, Liu Z, Zeng C. Clinicopathological features and outcome of antibody-negative anti-glomerular basement membrane disease. J Clin Pathol. 2019;72(1):31-37. [PubMed link]
  • Gulati K, McAdoo SP. Anti-Glomerular Basement Membrane Disease. Rheum Dis Clin North Am. 2018;44(4):651-673. [PubMed link]
  • Henderson SR, Salama AD. Diagnostic and management challenges in Goodpasture's (anti-glomerular basement membrane) disease. Nephrol Dial Transplant. 2018;33(2):196-202. [PubMed link]

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