Therapy of retroperitoneal fibrosis: Functional therapeutic outcome [Therapie der retroperitonealen Fibrose: Funktionelle Behandlungsergebnisse]

Background: The most frequent complication of retroperitoneal fibrosis (RPF) is an extrinsic compression of the ureter resulting in hydronephrosis. Because of the rareness of this uncommon chronic inflammatory disease, therapeutic results concerning the treatment of the obstructive uropathy are often known only for small study groups. Material and methods: The Else Kröner-Fresenius registry is a nationwide register of patients suffering from RPF in which all disease-related data are prospectively recorded. We performed a retrospective analysis of treatment outcome of different therapy strategies for all patients who underwent standardized treatment in our department since 2007. Results: From April 2007 to January 2014 a total of 97 patients with RPF were treated in our department. The mean age was 54.9 years and the male to female ratio was 3.2:1 (74 males and 23 females). Assessment of 85 out of 97 cases revealed 78 patients and 118 renal units with hydronephrosis. Of these patients 46 received prednisolone, 35 patients tamoxifen and 4 patients a combination therapy. Successful removal of double J stents was possible in 49 out of the 78 cases (62.8%) and in 72 out of 118 renal units (61.0%). Of the patients 26.9% received final surgical treatment of ureter compression and 7.7% of patients were permanently treated with a double J stents. Patients treated with prednisolone were significantly younger (p=0.040) and decided for surgical therapy significantly earlier (p=0.041). Otherwise there were no differences in functional outcome of patients treated with either prednisolone or tamoxifen. Conclusion: Removal of double J stents was possible in 63% of patients regardless of the medicinal approach to therapy, which supports the strategy of using medicinal treatment before the application of surgical solutions. Nevertheless, surgical interventions were necessary in more than 25% of cases. © 2014, Springer-Verlag Berlin Heidelberg.