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Currently filtering by tag: Anti-GBM

Twitter Poll (April 28, 2021)

Linear GBM Poll
  Answer: 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...

Twitter Poll (December 11, 2019)

Anti-GBM disease, twitter poll, arkana laboratories
Answer: D Anti-GBM disease presents with rapidly progressive glomerulonephritis (GN) with or without pulmonary hemorrhage. On biopsy typical disease displays bright polytypic linear GBM staining for IgG by immunofluorescence and diffuse crescentic/necrotizing GN on light microscopy. Anti-glomerular basement membrane (GBM) nephritis is clinically and pathologically one of the most aggressive form of glomerulonephritis. Patients typically present with nephritic syndrome and rapidly progressive acute kidney injury. Lung involvement in the form of alveolar hemorrhage occurs in 34% to 62% of patients. Anti-GBM disease is due to circulating autoantibodies directed against cryptic epitopes in the NC1 domain of the alpha-3 chain of...

Twitter Poll (August 22, 2018)

ANSWER: D Anti-GBM disease is a rare small vessel vasculitis that affects glomerular and pulmonary capillaries. The majority of patients develop widespread glomerular crescent formation, presenting with features of rapidly progressive GN, and 40-60% will have concurrent alveolar hemorrhage

Anti GBM Disease

This biopsy from an 80-year-old male with acute kidney injury shows glomeruli with frequent rupture of the capillary loops and Bowman’s capsule, associated with fibrinoid necrosis of the glomerular tuft and cellular crescent formation (Fig 1). Of note, there are scattered multinucleated giant cells within the involved glomeruli. Immunofluorescence shows diffuse and global bright linear staining of the capillary loops for IgG (Fig 2), kappa and lambda. These findings are diagnostic of anti-glomerular basement membrane disease.  Multinucleated giant cells are significantly more common in anti-glomerular basement membrane disease when compared to ANCA-associated glomerulonephritis, and may serve as a marker of...

Anti-Glomerular Basement Membrane

A 19-year-old Caucasian male patient presented with acute renal failure. Serum creatinine was 4.9 mg/dL and he had hematuria and non-nephrotic range proteinuria. Anti-GBM serologic testing was positive. No ANCA detected. Figure 1 one shows a cellular crescent filling Bowman’s space. Figure 2 also shows a cellular crescent associated with a prominent break in Bowman’s capsule (red arrow). Immunofluorescence shows global linear IgG staining of the capillary walls (Figure 3).

Pushing Glass (March 14, 2017)

The patient is a 61-year-old female who presents with hematuria and a creatinine of 6.23 mg/dL (baseline creatinine of 1.17 one month ago). What is the most likely diagnosis? A. Pauci-Immune Crescentic Glomerulonephritis B. Anti-Glomerular Basement Membrane Disease C. Crescentic Fibrillary Glomerulonephritis. D. Lupus Nephritis E. Early Diabetic Glomerulopathy The answer is B: The biopsy shows linear glomerular basement membrane staining for IgG, kappa, and lambda and this staining pattern is diagnostic of anti-glomerular basement membrane disease. Anti-glomerular basement membrane disease has a bimodal distribution in the general population. Younger patients (in their 20s) usually present with both hemoptysis and...