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Source | Study | n | Study Patients | Outcome |
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Vaglio et al., Lancet, 2011 | Prednisone vs. tamoxifen (0.5 mg/kg/d) for 8 months | 36 | Patients in remission after 1 month of prednisone | Lower relapse rate in prednisone (17%) vs. tamoxifen (50%) group (p-value 0.04) |
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Van Bommel et al., Am J Kidney Dis, 2007 | Prednisone (60 mg/d for 6 wks, tapered for 3 months to 10 mg/d for 1 y) | 24 | Patients with first presentation of idiopathic RPF | High initial success rate (75%) with high recurrence rate (72% of successes) |
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Van Bommel et al., Ann Intern Med, 2006 | Tamoxifen (20 mg bid) | 19 | 37% idiopathic RPF; 63% secondary to atherosclerosis | 79% reported symptom improvement with 6% recurrence |
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Jois et al., Rheumatology, 2007 | Cyclophosphamide (1 g/2 wks, 6 pulses) plus mycophenolate mofetil [MMF], 2.5 g/d) with reduced prednisolone | 1 | Relapsing RPF previously treated with reduced dose prednisolone and methotrexate | Asymptomatic No recurrence at 15 months |
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Scheel et al., Ann Intern Med, 2011 | MMF (1g twice daily for 10-30 m) with prednisone(40 mg/d, tapered over 6 m) | 7 | 1 patient had known RPF risk factor (external beam radiation) | Idiopathic RPF patients had 16%-62% mass regression; 10/11 ureters not obstructed after stent removal |
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Binder et al., Ann Rheum Dis, 2012 | Cyclophosphamide plus low dose corticosteroid induction; long term azathioprine or methotrexate | 35 | All patients had chronic periaortitis | Significant reduction of transverse diameter of periaortic mantle Less time between insertion and removal of ureteral stents |
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Marcolongo et al., Am J Med, 2004 | Prednisone plus either azathioprine or cyclophosphamide for 6 m | 26 | All patients had idiopathic RPF | Treatment failure rate of ~1 per 100 patient-years (95% CI: 0.02 to 5 per 100 patient-years) |
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Alberici et al., Ann Rheum Dis, 2012 | Methotrexate (15-20 mg/wk) plus prednisone | 14 | All patients had relapsing idiopathic RPF | 79% in remission, fewer relapses in patients continuing treatment; Significant CRP and ESR reduction, no significant mean mass reduction |
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Maritati et al., Ann Rheum Dis, 2012 | Rituximab (375 mg/m2/wk for 4 wks) | 2 | Both patients had chronic periaortitis. One relapsing; other administered rituximab plus low dose prednisone | Remission achieved in both patients |
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