A Case of Wegener's Granulomatosis with Ocular Involvement

ABSTRACT

Wegener's granulomatosis is characterized by necrotizing vasculitis and granuloma formation in both upper and lower respiratory tracts and in kidney. Other organs are also involved frequently. Ocular manifestations are Ehown in about 50% of patients with it. We experienced a case of Wegener's granulomatcsis developed prcgressive paranasal sinusitis and rhinitis combined with orbital and ocular involvement shown proptosis of both eyes, necrotic change of cornea and anterior portion of sclera in left eye and so forth. The diagnosis was confirmed by clinical findings and histopathologic features of tissue biopsy. The histopathology from periccular area of left eye had the typical findings of Wegener's granulomatosis with necrotizing vasculitis and granuloma formation. We had treated with oral cyclophosphamide(30~120mg/day) and corticosteroid(betamethaEcne 3mg 1M) and performed the enucleation of left eye due to widespread necrosis. The general condition and ocular findings were moderately improved with medical treatment but the eventual visual acuity was zero in right eye.