Unusual Presentation of Granulomatosis With Polyangitis (Wegener's Granulomatosis)

Abstract

Objective: To present a case of unusual presentations of granulomatosis with polyangitis (Wegener’s granulomatosis) with parotid swelling and intractable otitis externa. Material and method: A 22-year-old male patient presented with left sided otitis externa with left sided parotid swelling and bilateral nasal obstruction for last two weeks. CT scan of paranasal sinuses showed homogenous mass in both maxillary antra and nasal cavities. FNAC from the parotid swelling was suggestive of granulomatous disease and endoscopic biopsy from the nasal mass showed features of granulomatosis with polyangitis (Wegener’s granulomatosis). CT scan of chest revealed multiple cysts within the lung parenchyma; urine examination showed RBC and pus cells. Renal biopsy showed focal segmental glomerulonephritis. c-ANCA was highly positive and thus the diagnosis of Wegener’s granulomatosis was made. Result: Patient received cyclophosphamide and prednisolone immunsuppressive therapy for one year, which showed marked clinical improvement. Conclusion: Granulomatosis with polyangitis (Wegener’s granulomatosis) is not an uncommon entity for otolarygologists. Its usual presentation mimics chronic rhinosinusitis; but presentations like otitis externa and parotid swelling are rare for this disease