Granulomatosis with Polyangitis (GPA)

A 43-year-old lady presenting with longstanding bilateral nasal obstruction, anosmia, bloody crusts and blood-stained nasal discharge. She also had bilateral hearing loss. She tried some topical steroid nasal sprays and saline nasal douches before without much improvement. The patient also gave a history of cough, haemoptysis and dyspnoea, particularly on exertion, as well as haematuria. External nose examination has shown a saddle nose deformity. On examination by anterior rhinoscopy, the nasal mucosa shows multiple areas of ulceration and granulations. On endoscopic examination, the nasal cavity was filled with multiple crusts, and some adhesions between the septum and lateral nasal wall could be seen. Both tympanic membranes were markedly retracted, showing a clinical picture of otitis media with effusion. Endoscopic examination of the larynx has shown subglottic stenosis. C- ANCA was positive. Kidney function tests were elevated. CT scan of nose and sinuses has shown non-specific mucosal thickening and evidence of bone destruction. Renal biopsy has shown granulomatous inflammation with vasculitis and necrosis.