Retroperitoneal Fibrosis: Challenge in Diagnosis and Treatment

Objective: Literature documented for the diagnosis and treatment of retroperitoneal fibrosis (RF) is still relatively limited and diverse, which makes it more challenging, especially in the Taiwanese population. This article was aimed to describe the clinical manifestations, laboratory results, diagnostic tool, and treatments in patients with RF from a single tertiary institute. Patients and Methods: The International Classification of Diseases, Ninth Revision code 594.3 was used to identify patients evaluated for RF between January 2005 and August 2015, at Taipei Veterans General Hospital. Medical records were reviewed, and clinical information was collected. RF was diagnosed on the basis of image findings, as well as clinical, pathological, and laboratory data. Different treatments were compared for their efficacy on the protection of renal function. Results: A total of 30 patients were included in the study. Twenty-three (76.7%) were male. The mean age at diagnosis was 65.9 +/- 16.37. Mean body mass index was 31.5 +/- 3.47. Biopsies were done in 13 patients (43.3%). Half of the patients had serum IgG4 test, and the mean value was 249.3 +/- 205.1 mg/dL. Bilateral hydronephrosis was noted in 19 (63.3%) patients and was associated with poorer renal function than unilateral hydronephrosis at baseline. Most (93.3%) of the patients had improved renal function. However, statistically significant improvement was noted in patients with bilateral hydronephrosis instead of unilateral one. Compared with endoscopic ureteral stenting, surgical correction tended to have more renal functional protection, with significant serum creatinine (P = 0.006) and estimated glomerular flow rate (P = 0.035) change. Conclusion: RF in Taipei Veterans General Hospital was diagnosed through image, with the assistance of serum IgG4 and biopsy. There was a posttreatment improvement of renal function, especially in those with bilateral hydronephrosis. Surgical correction tended to be more beneficial than endoscopic procedure.