Fibrose rétropéritonéale idiopathique : évolution à long terme du pronostic rénal dans une série rétrospective multicentrique de 30 cas

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 m² 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.