Idiopathic retroperitoneal fibrosis: clinicopathologic features and outcome analysis

Abstract

To describe clinical features and outcomes of 26 patients with idiopathic retroperitoneal fibrosis from a single center, we reviewed medical records of consecutive patients with idiopathic retroperitoneal fibrosis evaluated at our facility from January 1, 1998 to December 31, 2013 for clinical features, laboratory and radiographic findings, management, and outcomes. Twenty-six patients met criteria for idiopathic retroperitoneal fibrosis and were included in the study. Median age at diagnosis was 58 years; male–female ratio was 3.3:1.0. Median duration of symptoms was 7 weeks. Abdominal, flank, and/or low back pain were the most common presenting symptoms. Four patients (15 %) had associated autoimmune or fibrosing disorders. Baseline erythrocyte sedimentation rate was elevated in 17 (77 %) of 22 patients tested and C-reactive protein was elevated in 10 (56 %) of 18 patients tested. Hydronephrosis was present in 17 (68 %) patients; 8 (47 %) of 17 had bilateral hydronephrosis. Retroperitoneal mass biopsy was performed in 18 (69 %) patients. Two patients had idiopathic retroperitoneal fibrosis classifiable as IgG4-related disease. Therapy consisted of medications alone in 7 cases, surgical interventions alone in 7 cases, and a combination in 11 cases. One patient achieved remission with no treatment. Most patients treated medically received initial corticosteroids. Methotrexate (1 case), azathioprine (1 case), mycophenolate mofetil (1 case), and tamoxifen (5 cases) were used. No relapses occurred after a median 5-year follow-up. Two (8 %) patients died; five (19 %) developed cancer after diagnosis. In this series, we emphasize the importance of early diagnosis and therapy for overall favorable prognosis of idiopathic retroperitoneal fibrosis.