International Ophthalmology

, Volume 28, Issue 1, pp 35–46 | Cite as

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

  • Muna Ahmed
  • John H. Niffenegger
  • Frederick Albert Jakobiec
  • Yael Ben-Arie-Weintrob
  • Nicolette Gion
  • Sofia Androudi
  • Robert Folberg
  • Michael B. Raizman
  • Curtis E. Margo
  • Morton E. Smith
  • Ian W. McLean
  • James G. Caya
  • C. Stephen Foster
Original Paper


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.


ANCA Histopathology Limited Ophthalmic Wegener’s 


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Copyright information

© Springer Science+Business Media B.V. 2007

Authors and Affiliations

  • Muna Ahmed
    • 1
  • John H. Niffenegger
    • 2
  • Frederick Albert Jakobiec
    • 3
  • Yael Ben-Arie-Weintrob
    • 1
  • Nicolette Gion
    • 1
  • Sofia Androudi
    • 1
  • Robert Folberg
    • 4
  • Michael B. Raizman
    • 5
  • Curtis E. Margo
    • 6
  • Morton E. Smith
    • 7
  • Ian W. McLean
    • 8
  • James G. Caya
    • 9
  • C. Stephen Foster
    • 1
  1. 1.Massachusetts Eye Research & Surgery InstituteHarvard Medical SchoolCambridgeUSA
  2. 2.Sarasota Retina InstituteSarasotaUSA
  3. 3.Massachusetts Eye & Ear InfirmaryBostonUSA
  4. 4.University of Illinois at ChicagoChicagoUSA
  5. 5.New England Eye CenterTufts University School of MedicineBostonUSA
  6. 6.University of South FloridaTampaUSA
  7. 7.Washington University School of MedicineSaint LouisUSA
  8. 8.Armed Forces Institute of PathologyWashingtonUSA
  9. 9.Medical Assessment and ConsultationCross PlainsUSA

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