Idiopathic retroperitoneal fibrosis: clinico-pathological characteristics

Idiopathic retroperitoneal fibrosis is a rare disease often causing obstructive uropathy because the fibrosis entraps the ureters. The retroperitoneal tissue is constituted by a fibrous component and a chronic inflammatory infiltrate with the former characterized by miofibroblasts. The infiltrate displayed perivascular. Last years, the immunosuppressive therapy and the decompression of obstructive renal failure have improved the prognostic. We reported 9 patients with Idiopathic retroperitoneal fibrosis in a Centre. We evaluate clinical symptoms at the presentation with the signs of renal and non-renal involvement. We evaluate the achieved therapy and the follow-up. All 9 patients had a radiological and/or histological diagnosis in the absence of malignancy, previous radiotherapy or gadolinium's administration with severe renal failure. The patients were treated with 1 mg/kg/day of prednisone three months and possibly the insertion of ureteral catheters. 89% patients have a high rate of initial success without renal failure, despite frequent disease relapse. A patient developed progression of the fibrosis.