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Combined prednisone and mycophenolate mofetil treatment for retroperitoneal fibrosis: a case series
Paul J Scheel1, Nancy Feeley, Stephen M Sozio
1The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA. PScheel1@jhmi.edu
Background:
Small case series suggest that a combination of mycophenolate 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 mycophenolate 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 fibrosis that warrants evaluation in randomized trials.
Primary Funding Source:
None.
Insights
This study found that combining prednisone and mycophenolate mofetil effectively treated retroperitoneal fibrosis. Most patients experienced symptom resolution and reduced mass size, with low recurrence rates.
Area of Science:
- Nephrology
- Immunology
- Rheumatology
Background:
- Retroperitoneal fibrosis (RPF) treatment options are limited.
- Small studies suggest mycophenolate mofetil and prednisone may be effective.
Purpose of the Study:
- To evaluate the outcomes of RPF patients treated with prednisone and mycophenolate mofetil.
Main Methods:
- Prospective case series of 28 RPF patients.
- Treatment: Prednisone (40 mg/d tapered over 6 months) and mycophenolate mofetil (2000 mg/d).
- Mean follow-up of 1012 days.
Main Results:
- Complete resolution of systemic symptoms in all patients.
- 89% showed ≥25% reduction in periaortic mass.
- Normalization of inflammatory markers and creatinine; low recurrence (2/28 patients).
Conclusions:
- Combined prednisone and mycophenolate mofetil therapy is a potentially effective treatment for RPF.
- Further evaluation in randomized trials is warranted.
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