title: IgG4-related systemic sclerosing disease of the ocular adnexa: a potential mimic of ocular lymphoma.
abstract: IgG4-related sclerosing disease has been described in the orbit and ocular adnexa. Of 164 biopsies of the ocular region for suspected lymphoma, we identified 6 cases of IgG4 disease, 4 of which were previously unrecognized. All 6 cases demonstrated increased plasma cells in a background of sclerosis and increased absolute numbers of IgG4-expressing cells. Our results confirm the difficulty in diagnosing IgG4-related sclerosing disease in the ocular region. Based on the findings, we suggest that specimens from biopsies of the eye and ocular adnexa for which a definitive diagnosis of lymphoma is not established undergo further workup for IgG and IgG4, particularly if increased plasma cells and sclerosis are present. When IgG4-expressing plasma cells account for greater than 50% of IgG-expressing plasma cells, a diagnosis of IgG4 disease should be considered. Timely recognition would benefit patients by allowing appropriate management with corticosteroid therapy and avoiding more aggressive or unnecessary therapeutic options.
authors.affiliation: Department of Pathology, Stanford University School of Medicine, CA 94305, USA.
authors.forename: Jason R
authors.initials: JR
authors.lastname: Karamchandani
authors.forename: Sheren F
authors.initials: SF
authors.lastname: Younes
authors.forename: Roger A
authors.initials: RA
authors.lastname: Warnke
authors.forename: Yasodha
authors.initials: Y
authors.lastname: Natkunam
mesh_terms: D000328:Adult; D000368:Aged; D003937:Diagnosis, Differential; D005128:Eye Diseases / Q000276:immunology / Q000473:pathology*; D005134:Eye Neoplasms / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008223:Lymphoma / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000276:immunology / Q000473:pathology*
chemical_list: D007074:Immunoglobulin G