title: [Retroperitoneal fibrosis in adults: Main characteristics and relevance of the diagnostic procedures based on a retrospective multicenter study on 77 cases].
abstract: BACKGROUND
  Retroperitoneal fibrosis (RPF) is a rare disorder characterized by the sheathing of retroperitoneal structures by fibro-inflammatory process. It can be either isolated or associated with an underlying disease or condition. In the absence of consistent and consensual approach, the objective of this study was to assess the relevance of diagnostic tests performed during the diagnostic work-up of RPF.
  
  
  METHODS
  Seventy-seven patients were included in this retrospective multicenter study. The diagnosis of RPF was defined by the presence of a thickened circumferential homogeneous tissue unsheathing the infrarenal aorta, excluding peri-aneurysmal fibrosis and a clear evidence of a cancer.
  
  
  RESULTS
  In 62 cases (80.5%), the RPF was considered as being primary or "idiopathic". Surgical (n=31) or CT-guided (n=9) biopsies of the RPF were performed in half of the patients showing some fibrotic or non-specific inflammatory lesions in 98% of cases. A bone marrow biopsy was performed in 23 patients leading to diagnosis of low grade B cell non-Hodgkin lymphoma in a single patient who also had a monoclonal gammopathy IgM. The systematic search for autoantibodies or serum tumor markers was of no diagnostic value.
  
  
  CONCLUSIONS
  Although the diagnostic procedure was heterogeneous, no cause or associated disease was found in the majority of cases of FRP in this series. In the absence of any clinical or paraclinical evidence suggesting an underlying disease or any atypical features at presentation, a number of non-invasive tests (autoantibodies, tumor markers, bone scintigraphy) and also more invasive diagnostic tests (bone marrow and RPF biopsies) seem of little relevance.
authors.affiliation: Service de médecine interne, université Francois-Rabelais, hôpital Bretonneau, CHU de Tours, 2, boulevard Tonnelé, 37044 Tours cedex 9, France. Electronic address: bertrand.lioger@univ-tours.fr.
authors.forename: B
authors.initials: B
authors.lastname: Lioger
authors.affiliation: Service de néphrologie, hôpital du Kremlin-Bicêtre, 78, rue du Général-Leclerc, 94275 Le Kremlin-Bicêtre, France.
authors.forename: Y
authors.initials: Y
authors.lastname: Yahiaoui
authors.affiliation: Service de médecine interne, hôpital Foch, 40, rue Worth, 92151 Suresnes cedex, France.
authors.forename: J-E
authors.initials: JE
authors.lastname: Kahn
authors.affiliation: Service de néphrologie et immunologie clinique, ITUN and Inserm UMR S-1064, CHU de Nantes, 44000 Nantes, France.
authors.forename: F
authors.initials: F
authors.lastname: Fakhouri
authors.affiliation: Service de néphrologie dialyse, hôpital André-Grégoire, 93100 Montreuil-sous-Bois, France.
authors.forename: X
authors.initials: X
authors.lastname: Belenfant
authors.affiliation: Service de médecine interne, hôpital Bichat, 46, rue Henri-Huchard, 75018 Paris, France.
authors.forename: T
authors.initials: T
authors.lastname: Papo
authors.affiliation: Service de médecine interne, université Francois-Rabelais, hôpital Bretonneau, CHU de Tours, 2, boulevard Tonnelé, 37044 Tours cedex 9, France.
authors.forename: J
authors.initials: J
authors.lastname: Magnant
authors.affiliation: Service de médecine interne, université Francois-Rabelais, hôpital Bretonneau, CHU de Tours, 2, boulevard Tonnelé, 37044 Tours cedex 9, France.
authors.forename: F
authors.initials: F
authors.lastname: Maillot
authors.affiliation: Service d'urologie, hôpital Henri-Mondor, AP-HP, 94000 Créteil cedex, France.
authors.forename: D
authors.initials: D
authors.lastname: Vordos
authors.affiliation: Service de médecine interne, centre de référence des cytopénies auto-immunes de l'adulte, université Paris-Est Créteil, hôpital Henri-Mondor, Assistance publique des Hôpitaux de Paris, 94000 Créteil, France.
authors.forename: B
authors.initials: B
authors.lastname: Godeau
authors.affiliation: Service de médecine interne, centre de référence des cytopénies auto-immunes de l'adulte, université Paris-Est Créteil, hôpital Henri-Mondor, Assistance publique des Hôpitaux de Paris, 94000 Créteil, France.
authors.forename: M
authors.initials: M
authors.lastname: Michel
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003937:Diagnosis, Differential; D019937:Diagnostic Techniques and Procedures / Q000592:standards; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis* / Q000453:epidemiology; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D007074:Immunoglobulin G
keywords: Fibrose rétropéritonéale; Fibrose rétropéritonéale maligne; IgG4-related disease; Maladie systémique associées aux IgG4; Malignant retroperitoneal fibrosis; Retroperitoneal fibrosis