[Idiopathic retroperitoneal fibrosis: a multicentric retrospective study of 30 French cases and follow-up of the renal function]

Rev Med Interne. 2014 Sep;35(9):570-6. doi: 10.1016/j.revmed.2013.11.006. Epub 2013 Dec 6.
[Article in French]


Purpose: Idiopathic retroperitoneal fibrosis (IRF) is an inflammatory disorder, affecting the aorta and the surrounding vessels and tissues. The prognosis is mainly driven by the risks of chronic kidney disease and relapse. Our aim was to assess the prevalence of chronic kidney disease at follow-up.

Methods: We retrospectively reviewed the medical records of patients diagnosed for IRF in Seine-Saint-Denis (France) between 1987 and 2011. We collected informations about presentation, radiologic findings and follow-up. Diagnosis of IRF was confirmed when all the following criteria were met: infiltration of the infrarenal aorta or iliac vessels, absence of aneurysmal dilation, lack of clinical suspicion of malignancy.

Results: Thirty patients were identified, with a male/female ratio of 4.9. Mean age was 55±13 years old. The mean creatinine clearance was 66 mL/min/1.73 m(2) and the mean CRP was 45±36 mg/L. In 24 (80%) patients, the location of IRF was periaortic and periiliac. Eleven patients (37%) underwent a diagnostic biopsy, and 14 (47%) required an ureteral procedure. A mean follow-up of 63 months was available for 29 patients: 69% relapsed, 7 developed chronic renal disease (24%), and one died of urinary sepsis. Older age (P=0.023), diabetes (P=0.007), and initial renal insufficiency (P=0.05) were associated with a risk of chronic renal insufficiency.

Conclusion: The high frequency of relapses and chronic renal disease emphasizes the need of close follow-up in patients diagnosed with IRF.

Keywords: Acute renal insufficiency; Chronic renal insufficiency; Fibrose rétropéritonéale; Insuffisance rénale aiguë; Insuffisance rénale chronique; Retroperitoneal fibrosis.

Publication types

  • English Abstract
  • Multicenter Study

MeSH terms

  • Adult
  • Aged
  • Female
  • Follow-Up Studies
  • France / epidemiology
  • Humans
  • Kidney / physiopathology*
  • Kidney Failure, Chronic / diagnosis
  • Kidney Failure, Chronic / epidemiology
  • Kidney Failure, Chronic / etiology
  • Kidney Function Tests
  • Male
  • Middle Aged
  • Prognosis
  • Retroperitoneal Fibrosis / complications
  • Retroperitoneal Fibrosis / diagnosis*
  • Retroperitoneal Fibrosis / epidemiology
  • Retroperitoneal Fibrosis / physiopathology*
  • Retrospective Studies