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What is 13-year-old female child presented with recurrent sinusitis fever, ahralgia, respiratory distress, Hematuria and hypeension. Renal biopsy showed necrotizing granuloma. The anti proteinase-3 ANCA was positive. The most likely diagnosis is: and explain in detail?
Respiratory & Renal involvement along with positive c-ANCA (Anti Proteinase 3) indicates Wegener's Granulomatosis