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Twitter Poll (April 28, 2021)

Joel Murphy, MD
By Joel Murphy, MD

Apr 29, 2021

Linear GBM Poll

Answer: A, Linear GBM

 

Anti-GBM disease is classically characterized by bright linear staining with IgG by immunofluorescence. By light microscopy, anti-GBM disease typically demonstrates a diffuse necrotizing, crescentic glomerulonephritis. Occasionally, multinucleated giant cells may be seen within the glomerular crescents as well as periglomerular areas. The pathophysiology of the disease is most often due to an autoantibody to the noncollagenous 1 (NC1) domain of the alpha 3 chain of collagen IV, however occasionally antibodies to the NC1 domain of the alpha 5 chain may also be present. Of note, a major prognostic factor at the time of biopsy is the degree of cellular crescent formation as most patients with >75% crescent formation at biopsy are dialysis dependent within 3 months.

 

 

 

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