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| Wegeners Granulomatosis |
| Etiology Unknown. •Many of the patients will be positive for antineutrophilic cytoplasmic antibodies (ANCA) but it is not certain whether these antibodies are etiologic or markers of the disease. |
| Pathogenesis •The pathogenesis of Wegener's granulomatosis is unknown but is presumed to be immunologic •The disease responds to steroids and cyclophasphamide. •The role of anti-neutrophilic cytoplasmic antibodies is uncertain at this point. •Wegener's granulomatosis is not believed to be immune complex mediated., |
| Epidemiology •Peak incidence is the fifth decade of life. •Many of the patients will be positive for antineutrophilic cytoplasmic antibodies (ANCA) but it is not certain whether these antibodies are etiologic or markers of the disease. |
| General Gross Description •The kidneys are enlarged and pale and the cortices may show a petechial or flea-bitten appearance reflecting glomerular necrosis. There may also be hemorrhagic tracts corresponding to necrotizing activity involving arcuate and interlobular arteries. |
| General Microscopic Description •The glomeruli show necrotizing lesions of the tuft with crescent development. •The arterial vasculature may show focal areas of necrosis with associated thrombosis and surrounding hemorrhage and inflammation. •Direct immunofluorescence is generally negative, although a few fluorescent flecks may be seen (pauci-immune). •Electron microscopy is not revealing, showing little more than the necrotizing and proliferative features appreciable by light microscopy. |
| Clinical Correlation •The renal presentation is that of rapidly progressive glomerulonephritis, meaning that the patient presents with acute renal failure and the disease process has targeted the glomerular compartment (proteinuria, active urine sediment with red cells and red cell casts) •The patient has pulmonary manifestations of disease with nodular to cavitary infiltates on chest Xray and pulmonary hemorrhage (hemoptysis). •Some patients have chronic sinusitis and nasopharyngeal disease. •Many of the patients (90%) will be positive for antineutrophilic cytoplasmic antibodies (ANCA) on serologic workup. |
| References • Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 496-497 and p. 963. •Rose B. Renal Pathophysiology the essentials. Baltimore: Williams and Wilkins. 1994. Ch. 9. |
| Wegeners Granulomatosis |
| Synopsis by: Harold Yamase M.D. (T71200M01111)[220] |
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