Successful use of steroids and ureteric stents in 24 patients with idiopathic retroperitoneal fibrosis : a retrospective study

Background/Aims: Retroperitoneal fibrosis (RPF) is a chronic inflammatory disorder causing obstructive nephropathy and renal failure. We reviewed our management of this condition. Method: All patients with RPF treated at a single center over a 15-year period were identified. A full review of notes and computer records was undertaken. Results: Data was available on 27 patients, 3 of which were excluded from later analysis. Diagnosis was based on clinical history and cross-sectional imaging. Retroperitoneal biopsy was undertaken in 3 patients. 96% had significant renal impairment at presentation with a mean serum creatinine of 688 μmol/l. 46% required emergency hemodialysis. All patients were treated with a combination of ureteric stents and/or steroids with an excellent clinical response. The mean best creatinine reached by the cohort was 136 μmol/l, and renal function remained stable in the long term. No patients required chronic dialysis. Ureteric stents were removed within 12 months and low-dose steroids were continued for a mean of 34 months. Recurrent disease was observed in 25% of patients, who all responded well to further steroid therapy. Mean duration of follow-up was 76 months. Conclusions: RPF is very effectively treated by a combination of ureteric stents and steroids, with excellent long-term results using this approach. Continued follow-up is advised because of the possibility of recurrent disease.