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Shunichiro Ueda, Hiroshi Goto, Yoshihiko Usui, Keisuke Kimura, Kazuhiko Umazume, Jun Matsubayashi, Toshitaka Nagao; IgG4-related ophthalmic disease: A clinicopathological study of 40 cases. Invest. Ophthalmol. Vis. Sci. 2014;55(13):5444.
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The aim of this study is to clarify the clinical and histopathological features of IgG4-related ophthalmic disease (IgG4-ROD).
We reviewed the medical records of patients with IgG4-ROD diagnosed at Tokyo Medical University Hospital between 2002 and 2013. The age and sex of the patients as well as the IgG4 serum levels and the findings of imaging studies, histopathological assessments, flow cytometric studies, and immunoglobulin heavy chain rearrangement assessments were investigated. IgG4-ROD was diagnosed based on (1) the presence of a swelling, enlargement, or mass in any ocular adnexal tissue detected by imaging studies and (2) IgG4 serum levels > 135 mg/dl or (3) >40% of IgG-positive plasma cells being IgG4-positive and >50 cells/field when a biopsy sample was observed using a high-powered microscope.
Based on the above criteria, 40 patients were diagnosed with IgG4-ROD, of which 20 (50%) were women. The mean age of the patients was 58.4 years (age range, 27-81 years). Imaging studies showed infiltrative lesions in both (n = 30) or one (n = 3) lacrimal gland, eyelid and extraocular muscles on both sides (n = 2), extraocular muscles on both sides (n = 2), the caruncles on both sides (n = 1), and as a unilateral orbital mass (n = 2). The average IgG4 serum levels were 613 mg/dL (range, 57-1,920 mg/dL). Twenty-seven patients underwent biopsy. Although histological examination revealed lymphoid follicles and mild fibrosis in many cases, strong fibrosis and unclear lymphoid follicles were also observed in a few cases. Immunohistochemically, average 61.5% of the IgG-positive plasma cells were IgG4-positive. No evidence of light chain restriction was observed in the flow cytometric study. Immunoglobulin heavy chain rearrangement was not detected but one patient.
The findings of the present study suggest that IgG4-ROD occurs in various regions of the ocular adnexa.
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