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Wegener's Granulomatosis - Coggle Diagram
Wegener's Granulomatosis
Multisystem disorder of unknown origin characterised by necrotising granulomatous inflammation and vasculitis of small and medium vessels
Pathophysiology
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Leading to the generation of micro abscesses, recruitment of monocytes and macrophages and lymphocytes to make granulomas
This results in the recruitment of more neutrophils and more activated neutrophils when there is NO INFECTION
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As neutrophil rolls along blood vessels before it migrates into tissues, autoantibodies bind to it and ACTIVATE NEUTROPHILS inappropriately
Clinical Presentation
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Renal disease causes rapidly progressive glomerulonephritis with crescent formation, proteinuria or haematuria
Starts with severe rhinorrhoea and subsequent nasal mucosal ulceration - characteristic 'saddle-nose' deformity
May be skin purpura or nodules, peripheral neuropathy, arthritis/arthralgia
Lesions involve upper respiratory tract, lungs and kidneys
Differential Diagnosis
Churg-Strauss syndrome - also affects small arteries, males in 40's, triad of rhinitis and asthma, eosinophilia and systemic vasculitis. High eosinophil count. ANCA positive.
Diagnosis
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Bloods
- c-ANCA is positive
- Elevated PR3 antibodies
- Raised ESR and CRP
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