Long-term outcome of idiopathic retroperitoneal fibrosis treated with surgical and/or medical approaches.

BACKGROUND: Retroperitoneal fibrosis is a severe disease that affects the ureters, causing renal insufficiency in three-quarters of patients. The optimal treatment is far from being established.

METHODS: Seventeen patients with idiopathic retroperitoneal fibrosis and ureteral entrapment followed in our unit for at least 1 year were selected for this study. At presentation 13 patients had renal insufficiency. All patients received steroids, associated with ureterolysis in five (group 1), with azathioprine in six (group 2) and with tamoxifen in six (group 3). Four patients of group 2 and five of group 3 received ureteral stenting or nephrostomy. There were no significant differences among the three groups or the clinical and biochemical characteristics at presentation.

RESULTS: All patients of groups 1 and 2 entered remission after therapy. One patient from group 3 did not respond to therapy. During a mean follow-up of 56 +/- 41 months, three patients (two from group 1, one from group 2, 18%) had a recurrence of the disease, which fully responded to retreatment in all three cases. At the last observation, all patients were alive; three patients (18%) had renal insufficiency, of them one from group 1 had to start dialysis 6 years after ureterolysis, one patient from group 2 and one from group 3 had serum creatinine of 1.5 mg/dl. Renal survival was 100% at 5 years and 80% at 10 years.

CONCLUSIONS: In most patients, each of the three different therapeutic approaches restored renal function and significantly reduced the fibrotic mass in the short-term and maintained stable serum creatinine in the long-term.