title: Bilateral Orbital IgG4-Related Disease with Systemic and Corneal Involvement Showing an Excellent Response to Steroid and Rituximab Therapy: Report of a Case with 11 Years Follow-Up.
abstract: IgG4-related disease is a newly recognized fibro-inflammatory condition. The purpose of this report is to present a patient with 11 years of follow-up, who revealed characteristic features of IgG4-related disease with systemic, orbital and corneal involvement and showed a favorable response to steroids and rituximab treatment.
authors.affiliation: a Department of Ophthalmology and Eye Hospital , Friedrich Alexander University of Erlangen-Nuremberg , Erlangen , Germany .
authors.forename: Agnes I
authors.initials: AI
authors.lastname: Berta
authors.affiliation: b Institute of Pathology, Friedrich Alexander University of Erlangen-Nuremberg , Erlangen , Germany , and.
authors.forename: Abbas
authors.initials: A
authors.lastname: Agaimy
authors.affiliation: a Department of Ophthalmology and Eye Hospital , Friedrich Alexander University of Erlangen-Nuremberg , Erlangen , Germany .
authors.forename: Joachim M
authors.initials: JM
authors.lastname: Braun
authors.affiliation: c Department of Rheumatology , Friedrich Alexander University of Erlangen-Nuremberg , Erlangen , Germany.
authors.forename: Bernhard
authors.initials: B
authors.lastname: Manger
authors.affiliation: a Department of Ophthalmology and Eye Hospital , Friedrich Alexander University of Erlangen-Nuremberg , Erlangen , Germany .
authors.forename: Friedrich E
authors.initials: FE
authors.lastname: Kruse
authors.affiliation: a Department of Ophthalmology and Eye Hospital , Friedrich Alexander University of Erlangen-Nuremberg , Erlangen , Germany .
authors.forename: Leonard
authors.initials: L
authors.lastname: Holbach
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy; D003320:Corneal Ulcer / Q000175:diagnosis* / Q000188:drug therapy; D004338:Drug Combinations; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D009916:Orbital Diseases / Q000175:diagnosis* / Q000188:drug therapy; D010195:Pancreatitis / Q000175:diagnosis* / Q000188:drug therapy; D010265:Paraproteinemias / Q000175:diagnosis* / Q000188:drug therapy; D010950:Plasma Cells / Q000473:pathology; D000069283:Rituximab / Q000627:therapeutic use*; D014057:Tomography, X-Ray Computed
chemical_list: D004338:Drug Combinations; D005938:Glucocorticoids; D007074:Immunoglobulin G; D007155:Immunologic Factors; D000069283:Rituximab
keywords: Bilateral IgG4-related orbital disease; peripheral ulcerative keratitis; sclerosing autoimmune pancreatitis; steroid and rituximab treatment