title: Orbital inflammation in IgG4-related sclerosing disease.
abstract: IgG4-related sclerosing disease is a recently described systemic inflammatory disease that should be considered when evaluating patients with nonspecific orbital inflammation (pseudotumor). Orbital biopsy is necessary to establish a diagnosis and demonstrates lymphoplasmacytic infiltration, fibrosis, obliterative phlebitis of medium and small veins, and variable degrees of eosinophilia. We report the clinical and histopathological findings of 2 patients who developed chronic orbital inflammation as a manifestation of IgG4-related sclerosing disease. The 2 cases illustrate the widely varying clinical characteristics of this elusive disease.
authors.affiliation: Cole Eye Institute, Cleveland Clinic, Cleveland, Ohio, USA.
authors.forename: Theodore
authors.initials: T
authors.lastname: Pasquali
authors.forename: Lynn
authors.initials: L
authors.lastname: Schoenfield
authors.forename: Steven J
authors.initials: SJ
authors.lastname: Spalding
authors.forename: Arun D
authors.initials: AD
authors.lastname: Singh
mesh_terms: D000293:Adolescent; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D001707:Biopsy, Needle; D005141:Eyelid Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006104:Granuloma, Plasma Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007150:Immunohistochemistry; D000069285:Infliximab; D008297:Male; D008875:Middle Aged; D009916:Orbital Diseases / Q000175:diagnosis* / Q000276:immunology* / Q000473:pathology; D010689:Phlebitis / Q000276:immunology; D011239:Prednisolone / Q000627:therapeutic use; D018570:Risk Assessment; D012507:Sarcoidosis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D012598:Sclerosis; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D007074:Immunoglobulin G; D011239:Prednisolone; D000069285:Infliximab