Retroperitoneal fibrosis: retrospective descriptive study on clinical features and management.
BACKGROUND
Retroperitoneal fibrosis (RPF) is a rare condition characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encases the ureters or abdominal organs. This study describes the clinical characteristics, diagnostic methods, and treatments and their effects on renal function.


METHODS
We conducted a retrospective analysis of patients diagnosed with RPF at Maisonneuve-Rosemont Hospital.


RESULTS
We identified 17 patients with RPF between 1998 and 2013. Eight patients were females (47%), and the mean age was 62±18 years. Eleven patients were idiopathic. Back pain was the most common symptom. All diagnoses were made based on the finding of a retroperitoneal mass on the computed tomography scan. Three patients had histological diagnosis of RPF and seven patients had unspecific changes on their biopsy. Twelve patients needed double-J stents, three patients had a temporary percutaneous nephrostomy, two patients had to have a nephrectomy for refractory ureteral obstruction, and one patient required hemodialysis. Ten patients with idiopathic RPF received medical treatment. In the treated group, only two patients had complete remission of the disease and five patients had improvement of their lesions. There were no deteriorations and only one relapse. Seven patients did not receive any treatment; two of them achieved complete remission, one of them deteriorated, and two of them had no changes.


CONCLUSIONS
Most of our cases of RPF were idiopathic. Almost all treated patients received prednisone and seemed to respond, at least partially. There was a lot of heterogeneity in patient management, which makes it difficult to compare treatment effects. However, treated patients seemed to have more favorable outcomes than those who were not.
Section of Nephrology.
Ann-Sophie
AS
Laroche
Section of Nephrology.
Robert Z
RZ
Bell
Section of Nephrology.
Sarah
S
Bezzaoucha
Section of Internal Medicine, Department of Medicine, Maisonneuve-Rosemont Hospital, University of Montreal, Montreal, QC, Canada.
Eva
E
Földes
Section of Nephrology.
Caroline
C
Lamarche
Section of Nephrology.
Michel
M
Vallée
hydronephrosis; renal failure; retroperitoneal fibrosis