Wegener’s Granulomatosis Presenting With an Isolated Paranasal Sinus Involvement in a Child

©2014 Turkish League Against Rheumatism. All rights reserved. Wegener’s granulomatosis (WG) is mostly seen in adults with ages between 20 and 50 without a sex predilection. It is a necrotizing granulomatous vasculitis mostly affecting the lungs (95%), paranasal sinuses (90%), kidneys (85%), nose or nasopharynx (65%), and joints (65%) accordingly.1-4 It is rarely seen in children.5,6 Childhood WG has many common findings with those of adult disease, especially respiratory tract and renal involvement. However, the disorder has a female predominance in children and less frequently involves joints and paranasal sinuses.7 Paranasal and thorax computed tomography (CT) can be used to support the diagnosis.8,9 The definite diagnosis is based on the presence of necrotizing granulomatous vasculitis in pulmonary, renal or paranasal sinus biopsy specimens.5 Here we report a previously healthy boy who presented with facial edema, a history of recurrent upper respiratory tract infection and recurrent sinusitis without any other complaints and findings, and who was later on diagnosed as having WG limited to the maxillary sinuses, without other systemic involvement.