title: Immunoglobulin G4-related orbital disease: report of two pediatric cases.
abstract: IgG4-related disease is a systemic fibro-inflammatory disorder mainly affecting the middle-aged and elderly population. IgG4-related orbital disease is very rare in childhood. We present here two children with IgG4-related orbital disease, one of whom responded well to prednisolone treatment while the other one was refractory to most immunosuppressive agents. It is important to treat patients at the early active stage of disease before fibrotic changes predominate. Thus, although rare, increased awareness of IgG4-related orbital disease in childhood may avoid delays in diagnosis and treatment.
authors.affiliation: Department of Paediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Ezgi Deniz
authors.initials: ED
authors.lastname: Batu
authors.affiliation: Department of Paediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Zehra Serap
authors.initials: ZS
authors.lastname: Arici
authors.affiliation: Department of Paediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Diclehan
authors.initials: D
authors.lastname: Orhan
authors.affiliation: Department of Ophtalmology Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Hayyam
authors.initials: H
authors.lastname: Kiratli
authors.affiliation: Department of Paediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Seza
authors.initials: S
authors.lastname: Özen
mesh_terms: D000293:Adolescent; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D015415:Biomarkers / Q000032:analysis; D001706:Biopsy; D002648:Child; D042241:Early Diagnosis; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis*; D007150:Immunohistochemistry; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009916:Orbital Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D011237:Predictive Value of Tests; D011239:Prednisolone / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D005938:Glucocorticoids; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D011239:Prednisolone