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M. Levi, S.R. Kodsi, S. Rubin, C. Lyons, R. Golden, S.E. Olitsky, S. Christiansen, D. Alcorn; Wegener's Granulomatosis in Children . Invest. Ophthalmol. Vis. Sci. 2006;47(13):587.
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Ocular involvement occurs in up to 50% of adults with Wegener’s. Wegener’s occurs infrequently in children. Only 12 cases of children with Wegener’s and ocular involvement are reported in the literature. We report the largest series to date of children whose ocular findings were the initial presenting signs of Wegener’s.
Retrospective chart review of 6 children diagnosed with Wegener’s granulomatosis at various institutions.
Presenting symptoms included red eyes (4), lid edema (3), diplopia (2), blurred vision (1). Anterior segment findings included iritis (3), perilimbal corneal infiltrates (1), scleritis (1), dacryoadenitis (1), conjunctivitis (2), lid edema (3), proptosis (2), limited motility (2). Posterior segment exam was normal in 5 patients. One patient presented with optic disc edema. Systemic findings included joint pain (2), weight loss (2), upper respiratory findings (2), otitis media (1), rash (1), vomiting plus abdominal pain (1). Diagnosis was made with ANCA testing alone (1), renal biopsy (2), orbital biopsy (1). One patient had positive ANCA confirmed with renal biopsy, and one patient had positive ANCA, positive renal biopsy, and positive nasal biopsy. All six patients were treated with steroids. Two patients were treated additionally with methotrexate, and one with cyclophosphamide. One patient received methotrexate, cyclophosphamide, and infliximab. All patients demonstrated improvement in their ocular signs and symptoms.
Presenting symptoms appear similar to those in adults and include decreased vision, redness, proptosis, scleritis, uveitis, and lid edema. Treatment improves ocular signs and symptoms, but often multiple chemotherapeutic agents are required.
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