Combined prednisone and mycophenolate mofetil treatment for retroperitoneal fibrosis: A case series

Background: Small case series suggest that a combination of my-cophenolate mofetil and prednisone may be an effective treatment for patients with retroperitoneal fibrosis. Objective: To describe the outcomes of adults with retroperitoneal fibrosis who received a combination of prednisone and mycophe-nolate mofetil. Design: Prospective case series of patients followed between 1 April 2005 and 1 July 2009. Setting: Single tertiary care facility. Patients: 28 patients with retroperitoneal fibrosis. Intervention: Prednisone, 40 mg/d, tapered over 6 months, and mycophenolate mofetil, 1000 mg twice daily, for a mean of 24.3 months. Measurements: Clinical course, laboratory assessment, and measurement of periaortic mass. Mean follow-up was 1012 days, and no patients were lost to follow-up. Results: Systemic symptoms resolved in all patients; 89% had a 25% or greater reduction in periaortic mass. Elevated erythrocyte sedimentation rate and serum creatinine level and decreased hemoglobin level normalized in all patients. Disease recurred in 2 of 28 patients. Limitation: This was a small case series. Conclusion: Combined prednisone and mycophenolate mofetil therapy is a potentially effective treatment for retroperitoneal fibro-sis that warrants evaluation in randomized trials. Primary Funding Source: None.