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Pathology - Anti-glomerular Basement Membrane Disease
Definition: A severe kidney illness induced by the production of autoantibodies against the glomerular basement membrane.
Epidemiology • Rare. The peak incidence occurs in adults aged 20 to 40 years.
Aetiology: Autoantibodies against the C-terminal region of type IV collagen.
Pathogenesis • Autoantibodies bind to the glomerular basement membrane, causing an autoimmune attack on the glomeruli.
Presentation: ARF caused by significant acute glomerular damage. Some individuals may experience pulmonary hemorrhage if the autoantibody also reacts with the alveolar basement membrane.
Light microscopy • Glomeruli exhibit segmental, necrotizing glomerulonephritis, with breaks in the glomerular basement membrane and production of crescents in the Bowman's space. • Unaffected segments of the glomeruli appear normal. • A lymphoplasmacytic immune cell infiltrate is found in the interstitium of afflicted glomeruli. immune fluorescence • IgG and C3 exhibit strong linear staining in the glomerular basement membrane. • Linear staining may also be observed in tubular basement membranes.
Electron microscopy • There are no immunological deposits present.
Prognosis: Prompt immunosuppressive therapy can limit disease activity, although renal damage may be irreversible.
Definition: A severe kidney illness induced by the production of autoantibodies against the glomerular basement membrane.
Epidemiology • Rare. The peak incidence occurs in adults aged 20 to 40 years.
Aetiology: Autoantibodies against the C-terminal region of type IV collagen.
Pathogenesis • Autoantibodies bind to the glomerular basement membrane, causing an autoimmune attack on the glomeruli.
Presentation: ARF caused by significant acute glomerular damage. Some individuals may experience pulmonary hemorrhage if the autoantibody also reacts with the alveolar basement membrane.
Light microscopy • Glomeruli exhibit segmental, necrotizing glomerulonephritis, with breaks in the glomerular basement membrane and production of crescents in the Bowman's space. • Unaffected segments of the glomeruli appear normal. • A lymphoplasmacytic immune cell infiltrate is found in the interstitium of afflicted glomeruli. immune fluorescence • IgG and C3 exhibit strong linear staining in the glomerular basement membrane. • Linear staining may also be observed in tubular basement membranes.
Electron microscopy • There are no immunological deposits present.
Prognosis: Prompt immunosuppressive therapy can limit disease activity, although renal damage may be irreversible.
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