Retroperitoneal fibrosis in the military hospital of Morocco.

Abstract

Retroperitoneal fibrosis (RPF) is a rare disease. It is characterized by the presence of fibro-inflammatory tissue involving retroperitoneal structures. The usual mode of presentation of this disease is with lumbar pain, kidney failure, and a biological inflammatory syndrome. The aim of our study is to describe the diagnostic, etiologic, therapeutic aspects and outcomes of RPF in a nephrology unit in Morocco. Twelve cases of RPF were included in our study. The mean age was 57 ± 10 years (32.70). Nine patients were male and three were female. Symptoms were highly variable, dominated by pain that was present in all patients. Venous compressive signs were described in four patients (33.3%), anuria in one patient (8.3%), and hematuria in two patients (16.6%). Laboratory examinations found an inflammatory syndrome in all patients and renal failure in nine patients (75%), with a mean serum creatinine at 35 mg/L ± 8.5. Diagnosis was suspected on the ultrasound data and confirmed by computed tomography or magnetic resonance imaging. RPF was idiopathic in nine patients (75%). It was secondary to aortic aneurysm in one patient (8.3%), Riedel's thyroiditis in one patient (8.3%), and drug induced in another patient (8.3%). All patients received surgical treatment along with corticosteroids. At six months, remission was achieved in nine patients, whereas three others had steroid resistance. These patients were treated by mycophenolate mofetil (MMF) at a dose of 2 g/day; two of them had intestinal intolerance to MMF and thus were treated by tamoxifen at a dose of 40 mg/day. At 24 months, they stabilized their renal function with incomplete regression of the fibrotic plate. No cases of recurrence were observed during the study period.