title: IgG4-related retroperitoneal fibrosis: a newly characterized disease.
abstract: Retroperitoneal fibrosis (RPF) is a rare disease characterized by chronic, nonspecific inflammatory and sclerotic or fibrotic tissue in the periaortic or periiliac retroperitoneum that encases adjacent structures. There will be a series of clinical manifestations once the proliferated fibrous tissues encase the abdominal aorta, iliac arteries and urinary duct. RPF is generally divided into two types: idiopathic retroperitoneal fibrosis (IRPF) without identified pathogenesis, making up about two-thirds of cases, and secondary retroperitoneal fibrosis. Recent studies on Immunoglobulin G4-related disease (IgG4-RD) reveal that abundant infiltration of IgG4 positive plasma cells is found in biopsies on the mass of RPF of some IRPF patients, which is identified as one spectrum of IgG4-RD and is named IgG4-related RPF. IgG4-related RPF is often misdiagnosed as retroperitoneal visceral malignancy and is treated with surgery. In addition, because of its good response to glucocorticoid, early detection and treatment is important. We review the definition, epidemiology, clinical features, diagnostic criteria, treatment and prognosis of IgG4-related RPF in this article to raise awareness of this newly characterized disease.
authors.affiliation: Gerontology Department, Tianjin Medical University General Hospital, Tianjin, China.
authors.forename: Linjuan
authors.initials: L
authors.lastname: Lian
authors.affiliation: Gerontology Department, Tianjin Medical University General Hospital, Tianjin, China.
authors.forename: Cong
authors.initials: C
authors.lastname: Wang
authors.affiliation: Gerontology Department, Tianjin Medical University General Hospital, Tianjin, China.
authors.forename: Jian-Li
authors.initials: JL
authors.lastname: Tian
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000818:Animals; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000276:immunology*; D015551:Autoimmunity / Q000187:drug effects; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000276:immunology*; D011237:Predictive Value of Tests; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000276:immunology*; D012187:Retroperitoneal Space / Q000473:pathology; D012307:Risk Factors; D009626:Terminology as Topic; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D005938:Glucocorticoids; D007074:Immunoglobulin G
keywords: IgG4-related disease; IgG4-related retroperitoneal fibrosis; retroperitoneal fibrosis