Retroperitoneal fibrosis: A retrospective review of clinical presentation, treatment and outcomes.

Retroperitoneal fibrosis (RPF) is a rare disease and has a high risk of developing chronic kidney disease (CKD). This retrospective study was carried out with the objective to study the epidemiological, clinical and therapeutic characteristics of RPF and identify the risk factors associated with its progression to CKD. All 30 cases (24 males and five females) of RPF admitted from January 1985 to December 2013 in the Military Hospital of Tunis were included in this study. The mean age was 50.5 years. Presentation was with lower back pain, acute renal failure and inflammatory syndrome in 93%, 56% and 43% of the cases, respectively. Sixteen patients (54%) had a creatinine clearance <60 mL/min at the time of diagnosis. Erythrocyte sedimentation rate and C-reactive protein of >30 mm/h and 10 mg/L were observed in 56% and 53% of cases, respectively. The abdominal computed tomography scan showed ureterohydronephrosis in 63% of the cases. Classes I, II and III according to Scheel's radiological classification were found in, respectively, 16%, 13% and 70% of cases. Biopsy of RPF was performed in 20% of the cases, and all showed an inflammatory infiltrate without signs of vasculitis. RPF was idiopathic in 85% of the cases. Oral corticosteroid therapy was started for all patients. After a mean follow-up time of 53.2 months, an initial favorable response was noted in 76% of the cases. Fifty-three percent of the patients have presented one or more relapses during follow-up and 20% progressed to CKD. Most relapses were successfully treated by corticosteroids; only five patients had required additional immunosuppressive therapy. Two patients died. Elevated creatinine at diagnosis, high urea, clearance of creatinine lower than 60 mL/min and the use of ureteral stents were identified as risk factors for development of CKD.