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| Anti-GBM Nephritis |
| Etiology •Unknown. •Hydrocarbon exposure may be etiologic in some cases. |
| Pathogenesis •This is an autoimmune disorder. •Anti-glomerular basement membrane antibodies are produced and complex with glomerular basement membranes and in some instances with the pulmonary alveolar basement membranes leading to their destruction., |
| Epidemiology •This disease is not common. •Most cases are young males between ages 20 to 30 years. •The male to female ratio is approximately 4:1. •Immunogenetics: HLA CR2; B7. |
| General Gross Description •The kidneys are enlarged and pale and the cortices may show a petechial or flea-bitten appearance. |
| General Microscopic Description •Anti-GBM nephritis is a fulminant and intensely destructive glomeruar disease. •The histologic hallmark of this form of destruction is crescent development. •Nearly all glomeruli show exuberant proliferation of epithelial cells in the urinary space. •On the PAM silver stain, the glomerular architecture is fragmented and capillary basement membranes and Bowman's capsules are discontinuous. •On direct immunofluorescence, the glomerular capillary basement membranes show smooth linear fluorescence for IgG and C3. •On electron microscopy the architectural damage and the proliferative features are seen but the glomerular basement membranes do not show recognizable immune complexes because the antibody-antigen complexes are so uniform distributed that they are sub-electron microscopic. |
| Clinical Correlation •The clinical presentation is that of rapidly progressive glomerulonephritis, meaning that the patient presents in acute renal failure and the disease process has targeted the glomerular compartment. •Patients with anti-GBM nephritis can also present with concomitant or subsequent pulmonary failure due to hemorrhage. |
| References • Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 947-948 and 717-718. •Rose B. Renal Pathophysiology the essentials. Baltimore: Williams and Wilkins. 1994. Ch. 9. |
| Anti-GBM Nephritis |
| Synopsis by: Harold Yamase M.D. (T71200M00111)[219] |
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