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

被引:35
作者
Ahmed M. [1 ]
Niffenegger J.H. [2 ]
Jakobiec F.A. [3 ]
Ben-Arie-Weintrob Y. [1 ]
Gion N. [1 ]
Androudi S. [1 ]
Folberg R. [4 ]
Raizman M.B. [5 ]
Margo C.E. [6 ]
Smith M.E. [7 ]
McLean I.W. [8 ]
Caya J.G. [9 ]
Foster C.S. [1 ]
机构
[1] Massachusetts Eye Research and Surgery Institute, Harvard Medical School, Cambridge, MA 02142
[2] Sarasota Retina Institute, Sarasota, FL 34239
[3] Massachusetts Eye and Ear Infirmary, Boston, MA
[4] University of Illinois at Chicago, Chicago, IL
[5] New England Eye Center, Tufts University School of Medicine, Boston, MA
[6] University of South Florida, Tampa, FL
[7] Washington University School of Medicine, Saint Louis, MO
[8] Armed Forces Institute of Pathology, Washington, DC
[9] Medical Assessment and Consultation, Cross Plains, WI
关键词
ANCA; Histopathology; Limited; Ophthalmic Wegener's;
D O I
10.1007/s10792-007-9109-y
中图分类号
学科分类号
摘要
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. © Springer Science+Business Media B.V. 2007.
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页码:35 / 46
页数:11
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