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Idiopathic retroperitoneal fibrosis (IRF) is a rare disease, characterised by a fibroinflammatory tissue surrounding the aortoiliac axis and frequently entrapping the ureters.1 ,2 Glucocorticoids effectively induce remission, but 24% to 72% of patients relapse, half of them repeatedly.3 ,4 Immunosuppressants and glucocorticoids are usually required in relapsing IRF but no studies are available.
In this prospective, open-label trial we enrolled 16 relapsing patients with IRF (July 2004 to April 2011) aged 18–85 years and with an estimated glomerular filtration rate (eGFR)>50 ml/min5 after ureteral decompression (if required), and treated them with methotrexate and prednisone for 12 months. Relapse was defined in case of mass enlargement, hydronephrosis, or disease-related symptoms associated with high inflammatory markers.3 Prednisone was given at 0.5–1 mg/kg/day depending on flare severity, tapered to 12.5–10 mg/day by month 3, 7.5–5 mg/day by month 6 and maintained at 5–2.5 mg/day until month 12. Methotrexate was given at 15–20 mg/week until month 12. After month 12, the clinician was free to continue or withdraw the treatment.
The primary endpoint was remission (at month 12) defined as a stable, reduced mass and absence of hydronephrosis, disease-related symptoms and normal inflammatory markers.3 Secondary endpoints were changes in erythrocyte sedimentation …