망막색소상피박리와 망막하출혈을 동반한 베게너씨 육아종증 1예

A Case of Wegener`s Granulomatosis with Retinal Pigment Epithelial Detachment and Subretinal Hemorrhage

Purpose: To report a case of very limited Wegener’s granulomatosis presenting with inflammation of the anterior segment, orbital pseudotumor, retinal pigment epithelial detachment, and subretinal hemorrhage. Methods: A 54-year-old man who was treated for pachymeningitis was seen in consultation for painful swelling of the left eye and periorbit, and visual loss of the right eye developed 2 days after admission. He was diagnosed with peripheral keratitis, scleritis, anterior uveitis, and pseudotumor of the left eye, and RPE detachment in the macula of the right eye. He was started on treatment with antibiotics, NSAIDs, and steroids. Twelve days later, he had no response to the treatment and developed otitis media and skin ulcer. A skin biopsy was performed. Results: Histological analysis showed a necrotizing inflammation of the small vessels in the skin lesion. The clinical and radiologic findings and the positive antineutrophil cytoplasmic antibody (ANCA) test were consistent with a diagnosis of Wegener’s granulomatosis. Treatment with oral steroids and cyclophosphamide improved the systemic and ocular symptoms dramatically. One year later, he developed choroidal neovascularization and subretinal hemorrhage in the right eye. He was started on oral steroids and cyclophosphamide, with a dramatic improvement in his general health and ocular state.