Retroperitoneal fibrosis or Ormond’s disease is a rare disorder, characterized by the development of extensive fibrosis throughout the retroperitoneum, typically centred over the anterior surface of the fourth and fifth lumbar vertebrae, resulting in entrapment and obstruction of retroperitoneal structures, especially the ureters, causing obstructive renal failure. In most cases, the etiology is unknown. But its occasional association with autoimmune diseases and its response to corticosteroids and immunosuppressive therapy suggest it’s probably immunologically mediated. Approximately 8% of cases are associated with a neoplastic process, including carcinoids. In some cases, an immune reaction to an external agent (e.g. drugs like beta-blockers, methysergide or methyldopa) may initiate fibrosis. Recently, investigators have coined the term hyper–immunoglobulin G4 (hyper-IgG4) syndrome because of elevated levels of IgG4 and abundant IgG4-rich plasma cells in the inflammatory infiltrate.
Optimal care requires an integrated approach among surgical and non-surgical options. The goals of therapy include preserving renal function, reducing the morbidity, excluding malignancy and suppressing inflammatory processes. Surgical ureterolysis has been the preferred primary mode of treatment because it allows biopsy specimens to be obtained while ureteral obstruction is relieved. Recently, the knowledge of a possible autoallergic etiology has prompted the use of corticosteroids and cytotoxic (mycophenolate mofetil, azathioprine) and antifibrotic (tamoxifen) agents.