Retroperitoneal fibrosis

Retroperitoneal fibrosis (RPF) is an uncommon collagen vascular disease of unclear aetiology. It is characterised by a chronic non-specific inflammation of the retroperitoneum, which can entrap and obstruct retroperitoneal structures, notably the ureters. Because of the protean manifestations of RPF, awareness of the disease is important. It is still not uncommon to detect RPF only after severe renal failure is present. This comprehensive review deals with the various aspects of RPF and tries to provide a framework for the diagnosis, treatment and follow-up of this intriguing condition. Although it may have various causes, chronic periaortitis appears to be an increasingly encountered form of secondary RPF in patients with advanced atherosclerosis. Irrespective of its cause, most cases of non-malignant RPF - if in the active 'cellular' stage - will respond to treatment with corticosteroids, thereby obviating the need for surgical treatment. The clinical and radiographic improvement seen after starting steroid therapy is often impressive and reassuring as to the diagnosis. Treatment with corticosteroids may also make aneurysmectomy, if indicated, feasible in the patient who presents with perianeurysmal fibrosis and renal failure. Accumulating data suggest alternative treatment strategies for steroid-resistant cases (i.e. intensive immunosuppression) or when steroids are not feasible (i.e. other forms of immunosuppression or hormonal treatment, particularly tamoxifen). Although early diagnosis and treatment provide excellent renal and patient outcome, long-term follow-up is mandatory in all cases.