Systemic Wegener's Granulomatosis with Severe Orbito-ocular Involvement

Orbito-ocular involvement in Wegener's granulomatosis is the result of both focal ischaemic vasculitis and granulomatous soft tissue inflammation. Necrotising keratoscleritis and orbital inflammation are two most characteristic ophthalmic presentations. We describe a 56-year-old man with systemic limited Wegener's granulomatosis, presenting with pulmonary fibrosis, pansinusitis and left mastoiditis. This was complicated by the development of a left severe necrotising anterior scleritis, peripheral ulcerative keratitis and orbital apex syndrome. Both c-ANCA and anti-PR3 were positive. Despite mainstay systemic immunosuppressive therapy with cyclophosphamide and prednisolone, the visual prognosis remained very poor. This was largely due to the presence of an irreversible ischaemic optic neuropathy, extensive corneoscleral melt and corneal neovascularisation. This case highlights the possible extent of orbital and ocular surface involvement in Wegener's granulomatosis, and hence the importance of vigilance by the physician.