May 2006
Volume 47, Issue 13
ARVO Annual Meeting Abstract  |   May 2006
Wegener's Granulomatosis in Children
Author Affiliations & Notes
  • M. Levi
    Ophthalmology, North Shore – Long Island Jewish Health, Great Neck, NY
  • S.R. Kodsi
    Ophthalmology, North Shore – Long Island Jewish Health, Great Neck, NY
  • S. Rubin
    Ophthalmology, North Shore – Long Island Jewish Health, Great Neck, NY
  • C. Lyons
    Ophthalmology, University of British Columbia, Vancouver, BC, Canada
  • R. Golden
    Ophthalmology, Children's Mercy Hospital, Kansas City, MO
  • S.E. Olitsky
    Ophthalmology, Children's Mercy Hospital, Kansas City, MO
  • S. Christiansen
    Ophthalmology, University of Minnesota, Minneapolis, MN
  • D. Alcorn
    Ophthalmology, Stanford University, Stanford, CA
  • Footnotes
    Commercial Relationships  M. Levi, None; S.R. Kodsi, None; S. Rubin, None; C. Lyons, None; R. Golden, None; S.E. Olitsky, None; S. Christiansen, None; D. Alcorn, None.
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science May 2006, Vol.47, 587. doi:
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    • Get Citation

      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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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose: : 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.

Methods: : Retrospective chart review of 6 children diagnosed with Wegener’s granulomatosis at various institutions.

Results: : 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.

Conclusions: : 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.

Keywords: autoimmune disease 

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