title: Retroperitoneal fibrosis: single-centre experience from 1992 to 2010, current status of knowledge and review of the international literature.
abstract: OBJECTIVE
  The aims of this study were to investigate retroperitoneal fibrosis in a Spanish hospital and present a review of the international literature to attempt to elucidate a diagnostic and therapeutic approach to this unusual pathology.
  
  
  METHODS
  A database search was performed in the pathology department and in the documentation service using the key words "retroperitoneal fibrosis" and "Ormond's disease", limiting the search to the years 1990-2010. Cases in which secondary retroperitoneal fibrosis was considered were excluded. In addition, a PubMed literature search was performed using the terms "retroperitoneal fibrosis" and "Ormond's", limiting the search to 1985-2011.
  
  
  RESULTS
  Twenty-two patients were diagnosed with idiopathic retroperitoneal fibrosis (IRF) or Ormond's disease. The most common symptom at the time of diagnosis was flank pain. With regard to laboratory findings, five patients (22.7%) had anaemia and eight (36.3%) had renal failure. Computed tomography (CT) was performed in 20 patients (90.9%) and the most common finding observed was retroperitoneal mass. Eighteen patients were started on corticosteroids, in six cases in association with azathioprine. Three patients had recurrence at 12, 24 and 72 months, respectively, and 15 patients required emergency surgery. Nine open surgical procedures were performed.
  
  
  CONCLUSIONS
  At present, IRF is considered an autoimmune disease that presents with local and systemic signs and symptoms. CT and magnetic resonance imaging are the two tests of choice in IRF diagnosis and follow-up. [(18)F]Fluorodeoxyglucose positron emission tomography is starting to be used for assessment and treatment response. A combination of medical and surgical treatment is usually applied. It is essential to administer corticosteroids alone or in association with other immunosuppressive drugs such as azathioprine. Laparoscopic ureterolysis, or robotic ureterolysis, if available, is the technique of choice.
authors.affiliation: Urology Services.
authors.forename: Ignacio
authors.initials: I
authors.lastname: Gómez García
authors.forename: Angel
authors.initials: A
authors.lastname: Sánchez Castaño
authors.forename: Marta
authors.initials: M
authors.lastname: Romero Molina
authors.forename: Emilio
authors.initials: E
authors.lastname: Rubio Hidalgo
authors.forename: Natanael
authors.initials: N
authors.lastname: García Betancourth
authors.forename: Raquel
authors.initials: R
authors.lastname: Labra González
authors.forename: Antonio
authors.initials: A
authors.lastname: Sampietro Crespo
authors.forename: Fernando
authors.initials: F
authors.lastname: Álvarez Fernández
authors.forename: Ángel
authors.initials: Á
authors.lastname: Flores Herrero
authors.forename: Antonio
authors.initials: A
authors.lastname: Gómez Rodríguez
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D001379:Azathioprine / Q000627:therapeutic use*; D019468:Disease Management; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis* / Q000628:therapy*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D018666:Ureteroscopy / Q000379:methods*
chemical_list: D000305:Adrenal Cortex Hormones; D001379:Azathioprine