Diagnosis of limited ophthalmic Wegener granulomatosis: distinctive pathologic features with ANCA test confirmation.
OBJECTIVE
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.


CONCLUSIONS
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.
Ahmed
Muna
M
Massachusetts Eye Research & Surgery Institute, Harvard Medical School, 8th Floor, 5 Cambridge Center, Cambridge, MA 02142, USA.
Niffenegger
John H
JH
Jakobiec
Frederick Albert
FA
Ben-Arie-Weintrob
Yael
Y
Gion
Nicolette
N
Androudi
Sofia
S
Folberg
Robert
R
Raizman
Michael B
MB
Margo
Curtis E
CE
Smith
Morton E
ME
McLean
Ian W
IW
Caya
James G
JG
Foster
C Stephen
CS
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D003520:Cyclophosphamide / Q000627:therapeutic use; D003607:Dacryocystitis / Q000175:diagnosis* / Q000188:drug therapy; D004359:Drug Therapy, Combination; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009916:Orbital Diseases / Q000175:diagnosis* / Q000188:drug therapy; D015864:Panuveitis / Q000175:diagnosis* / Q000188:drug therapy; D011241:Prednisone / Q000627:therapeutic use; D015423:Scleritis / Q000175:diagnosis* / Q000188:drug therapy; D014057:Tomography, X-Ray Computed; D014792:Visual Acuity
D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone