Erratum to “Management of idiopathic retroperitoneal fibrosis from the urologist’s
perspective” by Surcel Cristian, Mirvald Cristian, Pavelescu Cristian, Gingu Constantin,
Carmen Savu, Emre Huri and Ioanel Sinescu, Therapeutic Advances in
Urology, 2015 April; 7(2): 85–99. doi: 10.1177/1756287214565637.
Erratum in
Ther Adv Urol. 2016.
Cristian Surcel [corrected to Surcel, Cristian]; Cristian, Mirvald [corrected to Mirvald,
Cristian]; Cristian, Pavelescu [corrected to Pavelescu, Cristian]; Constantin, Gingu
[corrected to Gingu, Constantin]; Carmen, Savu [corrected to Savu, Carmen]; Sinescu, Ioanel
[corrected to Sinescu, Ioanel].
Abstract
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.
Keywords: differential diagnosis, idiopathic retroperitoneal fibrosis, medical
treatment, retroperitoneal biopsy, surgical treatment, ureterolysis
