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


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.


Corticosteroids Hydronephrosis Idiopathic retroperitoneal fibrosis Renal failure 


  1. 1.
    Vaglio A, Salvarani C, Buzio C (2006) Retroperitoneal fibrosis. Lancet 367:241–251PubMedCrossRefGoogle Scholar
  2. 2.
    Koep L, Zuidema GD (1977) The clinical significance of retroperitoneal fibrosis. Surgery 81:250–257PubMedGoogle Scholar
  3. 3.
    Wu J, Catalano E, Coppola D (2002) Retroperitoneal fibrosis (Ormond's disease): clinical pathologic study of eight cases. Cancer Control 9:432–437PubMedGoogle Scholar
  4. 4.
    Vaglio A, Corradi D, Manenti L et al (2003) Evidence of autoimmunity in chronic periaortitis: a prospective study. Am J Med 114:454–462PubMedCrossRefGoogle Scholar
  5. 5.
    Okada H, Takahira S, Sugahara S et al (1999) Retroperitoneal fibrosis and systemic lupus erythematosus. Nephrol Dial Transplant 14:1300–1302PubMedCrossRefGoogle Scholar
  6. 6.
    Ohara H, Nakazawa T, Ando T, Joh T (2007) Systemic extrapancreatic lesions associated with autoimmune pancreatitis. J Gastroenterol 42(Suppl 18):15–21PubMedCrossRefGoogle Scholar
  7. 7.
    Kamisawa T, Funata N, Hayashi Y et al (2003) Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut 52:683–687PubMedCrossRefGoogle Scholar
  8. 8.
    Hamano H, Kawa S, Ochi Y et al (2002) Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet 359:1403–1404PubMedCrossRefGoogle Scholar
  9. 9.
    Baker LR, Mallinson WJ, Gregory MC et al (1987) Idiopathic retroperitoneal fibrosis. A retrospective analysis of 60 cases. Br J Urol 60:497–503PubMedCrossRefGoogle Scholar
  10. 10.
    van Bommel EF, van Spengler J, van der Hoven B, Kramer P (1991) Retroperitoneal fibrosis: report of 12 cases and a review of the literature. Neth J Med 39:338–345PubMedGoogle Scholar
  11. 11.
    Kermani TA, Crowson CS, Achenbach SJ et al (2011) Idiopathic retroperitoneal fibrosis: a retrospective review of clinical presentation, treatment, and outcomes. Mayo Clin Proc 86(4):297–303PubMedCrossRefGoogle Scholar
  12. 12.
    Marcolongo R, Tavolini IM, Laveder F et al (2004) Immunosuppressive therapy for idiopathic retroperitoneal fibrosis: a retrospective analysis of 26 cases. Am J Med 116:194–197PubMedCrossRefGoogle Scholar
  13. 13.
    Gómez García I, Sánchez Castaño A, Romero Molina M et al (2012) Retroperitoneal fibrosis: single-centre experience from 1992 to 2010, current status of knowledge and review of the international literature. Scand J Urol. doi:10.3109/00365599.2012.747564
  14. 14.
    Uibu T, Oksa P, Auvinen A et al (2004) Asbestos exposure as a risk factor for retroperitoneal fibrosis. Lancet 363:1422–1426PubMedCrossRefGoogle Scholar
  15. 15.
    Parums DV, Brown DL, Mitchinson MJ (2004) Serum antibodies to oxidized low-density lipoprotein and ceroid in chronic periaortitis. Arch Pathol Lab Med 114:383–387Google Scholar
  16. 16.
    Vaglio A, Palmisano A, Corradi D, Salvarani C, Buzio C (2007) Retroperitoneal fibrosis: evolving concepts. Rheum Dis Clin North Am 33:803–817, vi-viiPubMedCrossRefGoogle Scholar
  17. 17.
    Martinez-Valle F, Solans-Laque R, Bosch-Gil J, Vilardell-Tarres M (2010) Aortic involvement in giant cell arteritis. Autoimmun Rev 9:521–524PubMedCrossRefGoogle Scholar
  18. 18.
    Vaglio A, Pipitone N, Salvarani C (2011) Chronic periaortitis: a large-vessel vasculitis? Curr Opin Rheumatol 23:1–6PubMedCrossRefGoogle Scholar
  19. 19.
    van Bommel EF, Siemes C, Hak LE, van der Veer SJ, Hendriksz TR (2007) Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone. Am J Kidney Dis 49:615–625PubMedCrossRefGoogle Scholar
  20. 20.
    Kardar AH, Kattan S, Lindstedt E, Hanash K (2002) Steroid therapy for idiopathic retroperitoneal fibrosis: dose and duration. J Urol 168:550–555PubMedCrossRefGoogle Scholar
  21. 21.
    Vaglio A, Palmisano A, Alberici F et al (2011) Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial. Lancet 378:338–346PubMedCrossRefGoogle Scholar

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

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