Successful treatment of idiopathic retroperitoneal fibrosis with combined immunosuppressive therapy

Katarina Obrenčević; Dejan Petrović; Predrag Aleksić; Marijana Petrović; Nemanja Rančić; Dragan Jovanović; Bojan Nikolić; Mirjana Mijušković; Neven Vavić; Ljiljana Ignjatović; Djoko Maksić

doi:10.2298/VSP170714008O

Katarina Obrenčević Military Medical Academy, Solid Organ Transplantation Center, Belgrade, Serbia
Dejan Petrović University of Kragujevac, Faculty of Medical Sciences, Department of Internal Medicine, Kragujevac, Serbia
Predrag Aleksić Military Medical Academy, Clinic for Urology, Belgrade, Serbia; University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, Serbia
Marijana Petrović Military Medical Academy, Clinic for Nephrology, Belgrade, Serbia; University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, Serbia
Nemanja Rančić Military Medical Academy, Centre for Clinical Pharmacology, Belgrade, Serbia; University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, Serbia
Dragan Jovanović Military Medical Academy, Clinic for Nephrology, Belgrade, Serbia; University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, Serbia
Bojan Nikolić Military Medical Academy, Institute of Radiology, Belgrade, Serbia
Mirjana Mijušković Military Medical Academy, Clinic for Nephrology, Belgrade, Serbia; University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, Serbia
Neven Vavić Military Medical Academy, Solid Organ Transplantation Center, Belgrade, Serbia
Ljiljana Ignjatović Military Medical Academy, Clinic for Nephrology, Belgrade, Serbia
Djoko Maksić Military Medical Academy, Clinic for Nephrology, Belgrade, Serbia; University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, Serbia

DOI: https://doi.org/10.2298/VSP170714008O

Keywords: retroperitoneal fibrosis, renal insufficiency, autoimmune diseases, adrenal cortex hormones, mycophenolic acid, remission induction, recurrence

Abstract

Abstract

Background/Aim. Idiopathic retroperitoneal fibrosis (IRF) is characterized by the fibroinflammatory periaortic tissue that affects the ureters, causing obstructive nephropathy and variable impairment of renal function. The findings strongly suggest an autoimmune etiology. The optimal treatment has not been established. The aim of this study was to analyze a long-term efficacy of combined corticosteroid therapy with mycophenolate mofetil (MMF) in the patients with IRF. Methods. We retrospectively followed 13 patients (8 males and 5 females) with IRF. All patients received corticosteroids and MMF. For the patients with severe renal failure, an initial ureteral decompression was made and prednisone was started orally 0.5 mg/kg with fast tapering. In cases with a mild renal failure corticosteroids were administrated as intravenous methylprednisolone pulses for 3 days, followed by oral prednisone. The dose of MMF was 1000 mg twice a day. MMF was stopped after 18 months and prednisone after 48 months. Results. Systemic symptoms resolved in all patients. Erythrocyte sedimentation (SE) rate declined from the mean of 67.6 to 26.3 mm/h and C-reactive protein (CRP) from the mean of 18.5 to 6.3 mg/L. In 7 out of 8 patients, the ureteral stents were successfully removed 13 weeks on average. Seven patients had 100% of reduction in the periaortic mass, and the average percent reduction was 76.9%. The kidney function improved and remained normal in 6 treated patients. In 4 patients a mild chronic renal failure remained due to afunction of one kidney. Three patients, with a prior chronic renal failure, did not get worse renal function. The disease recurred in 3 patients. There were no treatment side effects noted. Conclusion. Combination of corticosteroids and MMF is a potentially effective treatment in restoring the renal function and reducing the fibrotic tissue in the patients with idiopathic retroperitoneal fibrosis. It could prevent the need for ureteral stenting and surgery. Longer treatment may reduce a possibility of recurrence.

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