Systemic vasculitis characterised by necrotising granuloma in the upper respiratory tract, lower respiratory tract and necrotising glomerulonephritis

Affects small to medium sized vessels cANCA mediated

Pulmonary nodules (‘coin’ lesions) and infiltrates, purpura, oral ulceration and strawberry gingivitis, scleritis, conjunctivitis, progressive glomerulonephritis.

Rhinitis and epistaxis (often first sign), perforated nasal septum, malaise, arthralgia (60%), hearing loss, pulmonary haemorrhage and haemoptysis, cough, haematuria.

Bloods: FBC, cANCA

Urine: Proteinuria, haematuria, red cell casts

Imaging: Chest X-Ray shows lung shadows, pleural effusion, nodules, cavitation

Biopsy: Histology shows granulomatous change

Majority respond well, 20-40% suffer flare-ups

Renal failure, destruction of nasal septum, hearing loss