Management of Idiopathic Retroperitoneal Fibrosis from the Urologist's Perspective

Introduction: Idiopathic retroperitoneal fibrosis (IRF) is a rare disease characterized by a fibrotic reaction that affects retroperitoneal organs, especially the urinary tract. In this review we analyze the current imaging techniques, morphological characteristics, clinical aspects and therapeutic aspects of idiopathic retroperitoneal disease.

Methods: A PubMed search was conducted in December 2013 to find original articles, bibliographic reviews and series reports published in the past 15 years on idiopathic retroperitoneal fibrosis, its management and outcomes by combining terms like retroperitoneal fibrosis, periaortitis, treatment and autoimmune. A total of 89 articles were included in this review that referred strictly to IRF. We analyzed the imaging tools used for diagnostic and the decision making protocol used by physicians in the management of IRF.

Results: A computerized tomography (CT) scan represents the most commonly used imaging technique for diagnosis. Magnetic resonance imaging (MRI) is unable to differentiate more accurately between benign and malignant retroperitoneal fibrosis (RF) than a CT scan. Biopsy remains the most reliable diagnostic tool for IRF. However, the histological characteristics of IRF are not yet well-defined and the protocol for biopsy is not standardized in terms of template, number of biopsies and the immunohistochemical panel needed for positive diagnosis. The most common treatment reported is corticosteroid therapy alone or in combination with other immunosuppressants, whereas surgical treatment is reserved for severe cases. Indwelling ureteric stents represent the most common procedure for renal drainage, but their efficacy is questionable. Open ureterolysis remains the gold standard for surgical treatment, but its purpose is only to resolve the ureteric obstruction, not to treat the retroperitoneal fibrosis. Laparoscopic and robotic approaches have been reported to be feasible, but no prospective, comparative trials have been performed due to the rarity of the disease. Surgical technique is not standardized and the outcome of the treatment only evaluates the recovery of the renal function.

Conclusions: The imaging procedures available today are unable to accurately differentiate between idiopathic and malignant RF. A biopsy is mandatory to confirm the diagnosis, but there is no consensus regarding the template, timing and number of biopsies needed to exclude malignancy. Open ureterolysis represents the main surgical treatment for cases with severe IRF, and laparoscopic or robotic approach may be an option in selected cases. The recovery of the renal function is a surrogate for evaluating the success of the treatment. More clinical studies are needed in order standardize the protocol for diagnostic, treatment and follow up after medical or surgical management.