Medical management of retroperitoneal fibrosis

• Published in: Transactions of the American Clinical and Climatological Association 2012, Vol.123, p.283-291
• Main Authors: Scheel, Jr, Paul J, Sozio, Stephen M, Feeley, Nancy
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Anti-Inflammatory Agents - therapeutic use; Aortic Diseases - drug therapy; Disease Management; Dose-Response Relationship, Drug; Drug Therapy, Combination; Female; Follow-Up Studies; Humans; Male; Middle Aged; Mycophenolic Acid - analogs & derivatives; Mycophenolic Acid - therapeutic use; Prednisone - therapeutic use; Prospective Studies; Retroperitoneal Fibrosis - drug therapy; Retrospective Studies; Tertiary Care Centers; Treatment Outcome; Ureteral Obstruction - drug therapy
• ISSN: 0065-7778

Small series and case reports suggest that a combination of mycophenolate mofetil and prednisone is an efficatious and safe treatment for patients with retroperitoneal fibrosis. To describe the outcomes of patients with retroperitoneal fibrosis treated with a combination of prednisone and mycophenolate mofetil. Prospective, case series. 31 patients with retroperitoneal fibrosis. Single-center tertiary care facility. Prednisone 40 mg administered daily and tapered over 6 months and mycophenolate mofetil 1,000 mg given twice daily. Clinical course, laboratory assessment, measurement of periaortic mass. Systemic symptoms resolved in all patients. Eighty-nine percent of patients had a 25% or greater reduction in periaortic mass. Eighteen patients had 32 obstructed ureters. Thirty of these ureters were free of obstruction after an average of 513 days of therapy. Laboratory abnormalities of elevated erythrocyte sedimentation rate and serum creatinine and decreased hemoglobin levels normalized in all patients. Recurrent disease occurred in 2 of 28 patients. Combined prednisone and mycophenolate mofetil appears to be an effective therapeutic option for patients with retroperitoneal fibrosis.