Medical management of retroperitoneal fibrosis.

BACKGROUND

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.

OBJECTIVE

To describe the outcomes of patients with retroperitoneal fibrosis treated with a combination of prednisone and mycophenolate mofetil.

DESIGN

Prospective, case series.

PATIENTS: 31 patients with retroperitoneal fibrosis.

SETTING

Single-center tertiary care facility.

INTERVENTION: Prednisone 40 mg administered daily and tapered over 6 months and mycophenolate mofetil 1,000 mg given twice daily.

MEASUREMENT: Clinical course, laboratory assessment, measurement of periaortic mass.

RESULTS

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.

CONCLUSION

Combined prednisone and mycophenolate mofetil appears to be an effective therapeutic option for patients with retroperitoneal fibrosis.