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.
De Luca
S
S
Department of Surgical and Medical Disciplines, University Scholl of Medicine, Torino, Italy.
Terrone
C
C
Manassero
A
A
Rocca Rossetti
S
S
D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002908:Chronic Disease; D005260:Female; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D015796:HLA-B27 Antigen / Q000032:analysis; D006801:Humans; D016130:Immunophenotyping; D008137:Longitudinal Studies; D016131:Lymphocyte Subsets / Q000145:classification / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009852:Omentum / Q000637:transplantation; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D012185:Retroperitoneal Fibrosis / Q000188:drug therapy / Q000209:etiology* / Q000235:genetics / Q000276:immunology / Q000473:pathology / Q000601:surgery; D012598:Sclerosis; D014513:Ureter / Q000601:surgery; D014517:Ureteral Obstruction / Q000601:surgery
D000893:Anti-Inflammatory Agents; D015796:HLA-B27 Antigen; D011239:Prednisolone