Wegeners Granulomatosis

Wegeners Granulomatosis - Wegeners Granulomatosis JUN-KI...

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JUN-KI PARK Wegener’s Granulomatosis
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y Definition y History y Epidemiology y Clinical symptoms y Pathophysiology y Treatment
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Wegener granulomatosis (WG) is a complex, immune- mediated disorder, which along with microscopic polyangitis and Churg-Strauss syndrome, comprises a category of small vessel vasculitis related to antineutrophil cytoplasmic antibodies (ANCAs), characterized by a paucity of immune deposits.
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History y 1931: Klinger described a 70-year-old physician with constitutional symptoms, joint symptoms, proptosis, widespread upper respiratory tract inflammation, saddle nose deformity, glomerulonephritis, and pulmonary lesions. y 1936: Wegener reported three patients with similar clinical features and published his findings on their distinct clinical and histopathologic findings. Postulated ‘septic’ vasculitis. y 1954: Goodman and Churg : definitive description of WG characterized by triad of pathological features: 1) systemic necrotizing angiitis 2) necrotizing granulomatous inflammation of the respiratory tract 3) necrotizing glomerulonephritis
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Epidemiology of WG y Incidence in US: appx. 10 per million y Prevalence in US: appx. 3 per 100.000 persons. y Much higher prevalence as 80% of 5year survival with treatment. y Age specific increase: peak age 65-74 y More common in individuals of northern European descent y Slight male predominance
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Clinical Features of Wegener’s Granulomatosis Weight loss Malaise Fever Arthralgia Myalgia Upper respiratory tract disease Mouth ulcers CNS manifestation Glomerulonephritis progressing to renal failure: 70-80% with WG Lung involvement: pulmonary hemorrhage, granulomas General Major
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Renal Pathology y Early Fibrinoid necrosis of capillary loops y Later Diffuse proliferative, pauci-immune GN with basement membranes ruptures and cells in Bowman’s space with crescent formation. y End-Stage Sclerosed glomeruli Main pathologic difference between WG and MPA is the absence of granulomatous inflammation in MPA
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Anti-Neutrophil Cytoplasmic Antibodies y ANCAs are directed against antigens (PR3 (C-ANCA), MPO (P-ANCA)) present within the primary granules of neutrophils and monocytes; these antibodies produce tissue damage via interactions with primed neutrophils and endothelial cells.
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Risk Factors / Initating Events y Infection (Anti LAMP2 / mimicry) y Genetic factors (PTPN22) y Drugs (thiol, hydrazine containing compounds) y Alpha-1 antitrypsin deficiency (AAT is primary in vivo inhibitor of PR3) y Environmental Exposure (silica dust, mercury, lead)
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y Anti-Proteinase 3 (PR3) in 70 to 80% of patients y Anti-Myeloperoxidase (MPO) in approximately 10% y
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This note was uploaded on 12/24/2011 for the course STEP 1 taught by Professor Dr.aslam during the Fall '11 term at Montgomery College.

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Wegeners Granulomatosis - Wegeners Granulomatosis JUN-KI...

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