Orbital presentation of systemic vasculitis: a diagnostic and management challenge.

BACKGROUND Orbital involvement in Wegener's Granulomatosis (WG) is rare and has an overall good prognosis. CASE A 60-year-old hypertensive Indian female presented with vision loss and painful proptosis of left eye. Orbital incisional biopsy suggested necrotising small vessel inflammation. The saddle nose deformity and pedal nodulo- ulcerative lesions further consolidated the diagnosis of Wegener's granulomatosis. Systemic immunosuppressant provided remission and the only relapse was managed successfully with intravenous Rituximab. CONCLUSION Wegener's granulomatosis should be kept in the differential diagnosis of painful proptosis with a diffuse orbital mass in an elderly patient presenting with profound vision loss. Newer immune modulating agents are useful adjuncts in preventing relapses of this fatal disease.