ETIOPATHOGENESIS 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 r eport a series of 13 cases of IRF in order to evaluate the clinical, d iagnostic, laboratory, therapeutic and prognostic aspects. They also r eport a rare case (the ninth case reported in the literature) of multi focal fibrosclerosis. A possible genetic predisposition was studied by testing for the presence of immunophenotype HLA-B27; this test was po sitive in 44% of cases. A study of the immunological profile and lymph ocyte populations revealed the typical features of chronic immune dise ase. Experience with medical and surgical treatment is reported compar ing various procedures: ureterolysis followed by application of a vasc ularized omental flap over the ureter (without subsequent corticostero id therapy) gave the best results, with complete resolution of the sym ptoms and long-term successful alleviation of ureteric obstruction in 100% of patients, with a mean follow-up of 58 months.