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| Idiopathic RPGN |
| Etiology •Unknown. |
| Pathogenesis •Unknown., |
| Epidemiology •Affects all age groups. •There are no identifiable precipitating factors. |
| General Gross Description •The kidneys are enlarged and pale and the cortices may show a petechial or flea-bitten appearance. |
| General Microscopic Description •Idiopathic rapidly progressive glomerulonephritis is a fulminant and intensely destructive glomeruar disease. •The histologic hallmark of this form of destruction is widespread crescent development. •Nearly all glomeruli show exuberant proliferation of epithelial cells in the urinary space. •Direct immunofluorescence studies are negative. •On electron microscopy the architectural damage and the proliferative features are seen but the glomerular basement membranes do not show recognizable immune complexes. |
| Clinical Correlation •The clinical presentation is that of rapidly progressive glomerulonephritis, meaning that the patients present with acute renal failure and the disease process has targeted the glomerular compartment (proteinuria, urine sediment with red cells and red cell casts). •There is no involvement of organ systems other than the kidney. |
| References • Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 947-8. • Spargo BH. Renal biopsy pathology. New York: Wiley, 1980, pp. 177-190. |
| Idiopathic RPGN |
| Synopsis by: Harold Yamase M.D. (T46672M01110)[221] |
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