a young male develops serous otitis media of the left ear with cough, occassional hemoptysis, hematuria and epistaxis for 2months.hb is 7.5 g% with heavy protienuria, bp 170/100 mmhg, RA -ve and anca +ve the most probable diagnosis is

Wegener Granulomatosis * Triad of: -- Acute necrotizing granuloma of U or L -- Necrotizing or granulomatous vasculitis of small-medium vessels -- Renal disease: crescentic glomerulonephritis * C-ANCA in 95% of cases * Diagnosis -- cANCA * 95% sensitive in generalized disease * 60% sensitive in localized disease -- FBC, ESR, CRP, CXR, Urine analysis for casts -- Histology: Tissue from septum & all turbinates -- CT nose & paranasal sinuses * Non-specific mucosal thickening; bony destruction; new bone formation DIAGNOSTIC CRITERIA < ACR criteria (1990) > > 2 of 4 (Sensitivity 88%, Specificity 92%) 1. Nasal or oral inflammation 2. Abnormal chest radiograph: nodules, fixed infiltrates, or cavities 3. Urinary sediment: Microhematuria (>5 red blood cells /HPF) or RBC casts 4. Granulomatous inflammation on biopsy Histologic changes showing granulomatous inflammation within the wall of an aery or in the perivascular or extravascular area(aery ,aeriole) < CHCC nomenclature (1994) > 1. Granulomatous inflammation involving the respiratory tract 2. Vasculitis of small to medium-size vessels ref: harrisons 21st ed