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.
D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D008297:Male; D011241:Prednisone / Q000627:therapeutic use
D003520:Cyclophosphamide; D011241:Prednisone