title: Retroperitoneal fibrosis.
abstract: Retroperitoneal fibrosis (RPF) is a rare disease that is marked by systemic inflammation and the development of a periaortic fibroinflammatory mass. The fibroinflammatory infiltration can encase the abdominal aorta, ureters, and other abdominal organs. The clinical presentation often includes constitutional symptoms, abdominal pain, and signs of renal insufficiency or renal failure related to ureteral obstruction. Less frequently, RPF may present with vascular complications, such as venous thrombosis or claudication. The idiopathic form of RPF is most common but secondary forms have been described and are associated with malignancy and a variety of different medications. The pathophysiology is uncertain, but RPF has been linked with periaortitis and IgG4-related disease. Treatment centers on the relief of symptoms and complications associated with mass effects. Corticosteroids and other immunosuppressant therapies can improve constitutional symptoms, reduce infiltrate mass, and achieve disease remission, but a chronic relapsing course is not uncommon.
authors.affiliation: University of Wisconsin School of Medicine & Public Health, Madison, WI, USA.
authors.forename: Martha
authors.initials: M
authors.lastname: Tzou
authors.affiliation: Medical College of Wisconsin, Milwaukee, WI, USA.
authors.forename: David J
authors.initials: DJ
authors.lastname: Gazeley
authors.affiliation: University of Wisconsin School of Medicine & Public Health, Madison, WI, USA pjmason@medicine.wisc.edu.
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Mason
mesh_terms: D015746:Abdominal Pain / Q000175:diagnosis / Q000209:etiology; D001157:Arterial Occlusive Diseases / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D003287:Contrast Media; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007383:Intermittent Claudication / Q000175:diagnosis / Q000209:etiology; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use*; D011856:Radiographic Image Enhancement; D035583:Rare Diseases; D012185:Retroperitoneal Fibrosis / Q000150:complications / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology*; D018570:Risk Assessment; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome
chemical_list: D003287:Contrast Media; D011241:Prednisone
keywords: IgG4-related disease; claudication; idiopathic retroperitoneal fibrosis; peripheral artery disease