Associated Findings and Complications of Retroperitoneal Fibrosis in 204 Patients: Results of a Urological Registry

PURPOSE: We studied the frequency of associated findings and complications of retroperitoneal fibrosis in a large clinical collective of urological patients. MATERIALS AND METHODS: All urology departments in Germany were invited to participate in a registry of patients with retroperitoneal fibrosis, for which a data sheet with more than 200 questions was developed. RESULTS: As of March 2010 a total of 204 patients were registered. The male-to-female ratio was 2.1:1 and the average age at onset was 55.6 years. In 123 cases (60.3%) the diagnosis was confirmed by histopathology. Coexisting autoimmune diseases were found in 9.8% of patients, consisting mainly of thyroid disorders. Coexisting fibrosis was detected in 3.4% of the patients and 73.9% of those queried were active smokers. Of 176 patients for whom data on therapy were available 123 received monotherapy and 41 received combination therapy for a mean of 12.1 months (12 received none), while 87 underwent a total of 103 operative procedures. Hydronephrosis was the most frequent complication in 95.6% of patients. Atrophic kidney from undetected hydronephrosis appeared in 46 patients (22.5%) and in 4 bilateral damage necessitated dialysis. Complications from vascular obstruction were observed in 27.5% of patients. Large bowel obstruction requiring colostomy occurred in 4 patients (2.0%). CONCLUSIONS: Patients with retroperitoneal fibrosis often first present to urology departments upon referral for hydronephrosis. In this series at least 1 kidney appeared to be irreversibly damaged in more than 20% of patients. Urologists should be mindful of the role of smoking role as a risk factor, complications arising from vascular and large bowel obstructions, and the possible association of retroperitoneal fibrosis with autoimmune disease.