Clinical Rheumatology

, Volume 32, Issue 6, pp 889–893

Idiopathic retroperitoneal fibrosis: a clinicopathological study in 24 Spanish cases

  • Andreu Fernández-Codina
  • Fernando Martínez-Valle
  • Jesús Castro-Marrero
  • Inés DeTorres
  • Miquel Vilardell-Tarrés
  • José Ordi-Ros
Case Based Review

DOI: 10.1007/s10067-013-2225-3

Cite this article as:
Fernández-Codina, A., Martínez-Valle, F., Castro-Marrero, J. et al. Clin Rheumatol (2013) 32: 889. doi:10.1007/s10067-013-2225-3

Abstract

Idiopathic retroperitoneal fibrosis (IRPF) is a rare condition of unknown aetiology characterized by chronic non-specific inflammation of the retroperitoneum. The aim of this study is to describe, for the first time, the features of Spanish patients with IRPF. In this retrospective study, a clinical description was performed to examine the histopathological features, radiologic findings, laboratory data and treatment of a cohort of 24 IRPF Spanish patients who were admitted to Vall d’Hebron Hospital in Barcelona (Spain) between 1982 and 2009. Patients with secondary retroperitoneal fibrosis were excluded. Nineteen patients (79.1 %) were male, whereas 5 were female. The mean age at diagnosis was 51.8 ± 16.4 years. Pain (79.1 %) and hydronephrosis (70.8 %) were the most common clinical manifestations. Abdominal computed tomography and ultrasonography were performed on all cases. Acute-phase reactants were elevated in most patients. Thirteen surgical biopsies were performed, and all were consistent with retroperitoneal fibrosis. Steroid therapy was given in 19 cases, and eight patients (42.1 %) required urethral catheterisation. Chronic renal failure (CRF) rate after 2 years of follow-up was 42.8 %, more frequently in patients with higher serum creatinine at diagnosis. IRPF in Spain is a rare condition affecting mainly middle-aged males, with pain and hydronephrosis as the most frequent manifestations. Steroids are the mainstay treatment, and CRF is the main sequel. An earlier diagnosis and uniform treatment protocols could prompt lower CRF rates.

Keywords

CorticosteroidsHydronephrosisIdiopathic retroperitoneal fibrosisRenal failure

Copyright information

© Clinical Rheumatology 2013

Authors and Affiliations

  • Andreu Fernández-Codina
    • 1
    • 2
  • Fernando Martínez-Valle
    • 1
    • 2
    • 4
  • Jesús Castro-Marrero
    • 2
  • Inés DeTorres
    • 3
  • Miquel Vilardell-Tarrés
    • 1
    • 2
  • José Ordi-Ros
    • 1
    • 2
  1. 1.Systemic Autoimmune Disease Unit, Department of Internal MedicineHospital Universitari Vall d’Hebron, Universitat Autònoma de BarcelonaBarcelonaSpain
  2. 2.Systemic Autoimmune Disease Research Unit, Vall d’Hebron Institute of ResearchHospital Universitari Vall d’Hebron, Universitat Autònoma de BarcelonaBarcelonaSpain
  3. 3.Pathology DepartmentHospital Universitari Vall d’Hebron, Universitat Autònoma de BarcelonaBarcelonaSpain
  4. 4.Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina InternaHospital Universitari Vall d’HebronBarcelonaSpain