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Diagnosis of limited ophthalmic wegener granulomatosis: distinctive pathologic features with ANCA test confirmation

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Abstract

Purpose To describe the clinical and histopathologic finding of very limited ophthalmic Wegener granulomatosis (WG). Methods Thirteen patients with scleritis, orbitopathy, episcleritis, and panuveitis were studied. They presented without evidence of lung or kidney disease, though eight had sinus involvement. We reviewed the biopsies for histopathologic findings consistent with WG, and tested for antineutrophil cytoplasmic antibodies antineutrophil cytoplasmic antibody (ANCA). Results WG was suggested by granulomatous foci, collagen necrosis, neutrophils/nuclear dust, plasma cells and infiltrating eosinophils. Granular degeneration of the interstitial collagen; mummification of the collagen with disappearance of fibroblastic nuclei; and a polymorphous infiltrate exhibiting plasma cells, lymphocytes, neutrophils, and eosinophils within the epithelioid granulomas should suggest the diagnosis. ANCA test results supported the diagnosis of WG in all cases. Conclusion The described histologic characteristics are highly suggestive of WG. These findings along with clinical or laboratory findings, allow the diagnosis of very limited ophthalmic WG in the absence of systemic involvement.

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Authors and Affiliations

  1. Massachusetts Eye Research & Surgery Institute, Harvard Medical School, 8th Floor, 5 Cambridge Center, Cambridge, MA, 02142, USA

    Muna Ahmed, Yael Ben-Arie-Weintrob, Nicolette Gion, Sofia Androudi & C. Stephen Foster

  2. Sarasota Retina Institute, 3400 Bee Ridge Road, Sarasota, FL, 34239, USA

    John H. Niffenegger

  3. Massachusetts Eye & Ear Infirmary, Boston, MA, USA

    Frederick Albert Jakobiec

  4. University of Illinois at Chicago, Chicago, IL, USA

    Robert Folberg

  5. New England Eye Center, Tufts University School of Medicine, Boston, MA, USA

    Michael B. Raizman

  6. University of South Florida, Tampa, FL, USA

    Curtis E. Margo

  7. Washington University School of Medicine, Saint Louis, MO, USA

    Morton E. Smith

  8. Armed Forces Institute of Pathology, Washington, DC, USA

    Ian W. McLean

  9. Medical Assessment and Consultation, Cross Plains, WI, USA

    James G. Caya

Authors
  1. Muna Ahmed
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  2. John H. Niffenegger
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  3. Frederick Albert Jakobiec
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  4. Yael Ben-Arie-Weintrob
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  9. Curtis E. Margo
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  11. Ian W. McLean
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  12. James G. Caya
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  13. C. Stephen Foster
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Corresponding author

Correspondence to C. Stephen Foster.

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Ahmed, M., Niffenegger, J.H., Jakobiec, F.A. et al. Diagnosis of limited ophthalmic wegener granulomatosis: distinctive pathologic features with ANCA test confirmation. Int Ophthalmol 28, 35–46 (2008). https://doi.org/10.1007/s10792-007-9109-y

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  • DOI: https://doi.org/10.1007/s10792-007-9109-y

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