Wegener's granulomatosis in a 7 year old child

A 7 year old boy presented with a history of recurrent episodes of sinusitis and otitis. He developed paresis of the external muscle of his right eye with exophthalmos. A biopsy of his sinus mucosa demonstrated necrotizing granulomatous involvement of the membranes with the presence of multiple giant cells interspersed in zones of fibrosis. A diagnosis of Wegener's granulomatosis was entertained. However the absence of pulmonary and renal manifestations did not allow for a definitive diagnosis to be made. He later developed hematuria and a renal biopsy demonstrated a renal lesions consistent with Wegener's granulomatosis. He has responded well to Cyclophosphamide and Prednisone therapy.