title: IgG4-related retroperitoneal fibrosis: the first reported case in a Chinese population.
abstract: Immunoglobulin G4 (IgG4)-related sclerosing disease is a newly recognized clinicopathological entity characterized by lymphoplasmacytic infiltration and varying degrees of fibrosis in various organs, with abundant IgG4-positive plasma cells in tissues. Patients usually exhibit multisystem involvement and often respond well to steroid and immunosuppressive therapy. However, this disease has been rarely reported in a Chinese population. We herein report a case of IgG4-related sclerosing disease solely presenting with retroperitoneal fibrosis that was effectively treated with systemic steroid therapy. To the best of our knowledge, this is the first reported case of IgG4-related retroperitoneal fibrosis in a Chinese population.
authors.affiliation: Divisions of Allergy, Chi-Mei Medical Center, Tainan, Taiwan.
authors.forename: Yeong-Jang
authors.initials: YJ
authors.lastname: Lin
authors.forename: Pei-Chih
authors.initials: PC
authors.lastname: Chen
authors.forename: Hung-An
authors.initials: HA
authors.lastname: Chen
authors.forename: Chien-Feng
authors.initials: CF
authors.lastname: Li
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D044466:Asian People; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000208:ethnology / Q000276:immunology* / Q000473:pathology; D001707:Biopsy, Needle; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007150:Immunohistochemistry; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D012185:Retroperitoneal Fibrosis / Q000188:drug therapy / Q000208:ethnology / Q000276:immunology* / Q000473:pathology; D012598:Sclerosis; D013624:Taiwan; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents