Aetiopathogenesis and treatment of idiopathic retroperitoneal fibrosis

Idiopathic retroperitoneal fibrosis (IRF) is a rare urological disease, for which many pathogenic theories have been proposed. The authors report a series of 13 cases of IRF in order to evaluate the clinical, diagnostic, laboratory, therapeutic and prognostic aspects. They also report a rare case (the ninth case reported in the literature) of multifocal fibrosclerosis. A possible genetic predisposition was studied by testing for the presence of immunophenotype HLA-B27; this test was positive in 44% of cases. A study of the immunological profile and lymphocyte populations revealed the typical features of chronic immune disease. Experience with medical and surgical treatment is reported, comparing various procedures: ureterolysis followed by application of a vascularized omental flap over the ureter (without subsequent corticosteroid therapy) gave the best results, with complete resolution of the symptoms and long-term successful alleviation of ureteric obstruction in 100% of patients, with a mean follow-up of 58 months.