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Orbital Inflammation With IgG4-Positive Plasma Cells: Manifestation of IgG4 Systemic Disease.
MedLine Citation:
PMID:  21482868     Owner:  NLM     Status:  In-Data-Review    
OBJECTIVE: To describe clinical, radiographic, and morphologic findings in patients with IgG4-positive cells present on orbital biopsy specimens.
DESIGN: Retrospective review (from January 1, 1993, through December 31, 2006) of patients with orbital biopsy specimens that excluded lymphoma; comparison of patients with and without IgG4-positive cells on immunostaining.
RESULTS: Of 21 patients, 11 had increased IgG4-positive cells (defined as >10 cells on biopsy). Symptoms included eyelid or periocular swelling (8 patients) or proptosis (3 patients), with bilateral involvement in 6 patients. Computed tomographic imaging displayed lacrimal gland mass in 10 patients; 6 patients had lesions in other organs. Two patients had increased serum IgG4 levels. In 10 patients without IgG4-positive cells (≤10 cells on biopsy), 6 had proptosis, 1 had eyelid swelling, 2 had eyelid mass, and 1 had diplopia, all unilateral. None had systemic symptoms. Patients with IgG4-positive cells had longer symptom duration, and their biopsy specimens showed more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils.
CONCLUSIONS: The clinical appearance, high incidence of bilateral disease, association with lesions in other organs, and increased IgG4 serum levels in some patients-with an increased number of IgG4-positive cells in the biopsy specimen, which shows more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils-indicate that these patients have an orbital manifestation of IgG4-associated systemic disease.
José Antonio Plaza; James A Garrity; Ahmet Dogan; Anuradha Ananthamurthy; Thomas E Witzig; Diva R Salomão
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Archives of ophthalmology     Volume:  129     ISSN:  1538-3601     ISO Abbreviation:  Arch. Ophthalmol.     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-04-12     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7706534     Medline TA:  Arch Ophthalmol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  421-8     Citation Subset:  AIM; IM    
Department of Pathology, Mayo Clinic, 200 First St SW, Rochester, MN 55905.
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