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Original Research |

Combined Prednisone and Mycophenolate Mofetil Treatment for Retroperitoneal Fibrosis: A Case Series

Paul J. Scheel Jr., MD; Nancy Feeley, CRNP; and Stephen M. Sozio, MD, MHS
[+] Article and Author Information

From The Johns Hopkins University School of Medicine, Baltimore, Maryland.


Potential Conflicts of Interest: Disclosures can be viewed at www.acponline.org/authors/icmje/ConflictOfInterestForms.do?msNum=M10-1908.

Reproducible Research Statement:Study protocol: Available from Dr. Scheel (e-mail, PScheel1@jhmi.edu). Statistical code and data set: Not available.

Requests for Single Reprints: Paul J. Scheel Jr., MD, Division of Nephrology, The Johns Hopkins University School of Medicine, 1830 East Monument Street, Room 416, Baltimore, MD 21205; e-mail, PScheel1@jhmi.edu.

Current Author Addresses: Dr. Scheel and Ms. Feeley: Division of Nephrology, The Johns Hopkins University School of Medicine, 1830 East Monument Street, Room 416, Baltimore, MD 21205.

Dr. Sozio: Division of Nephrology, Johns Hopkins Bayview Medical Center, 301 Mason F. Lord Drive, Suite 2515, Baltimore, MD 21224-2780.

Author Contributions: Conception and design: P.J. Scheel.

Analysis and interpretation of the data: P.J. Scheel, S.M. Sozio.

Drafting of the article: P.J. Scheel.

Critical revision of the article for important intellectual content: P.J. Scheel, S.M. Sozio.

Final approval of the article: P.J. Scheel, S.M. Sozio.

Statistical expertise: S.M. Sozio.

Collection and assembly of data: P.J. Scheel, N. Feeley.


Ann Intern Med. 2011;154(1):31-36. doi:10.7326/0003-4819-154-1-201101040-00005
Text Size: A A A

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.

Figures

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Appendix Figure 1.
Study flow diagram.

MMF = mycophenolate mofetil; RPF = retroperitoneal fibrosis.

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Figure 1.
Representative baseline and follow-up computed tomography scan.
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Figure 2.
Results of parametric testing of change in laboratory values.

To convert creatinine values to µmol/L, multiply by 88.4. ESR = erythrocyte sedimentation rate.

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Appendix Figure 2.
Results of nonparametric testing of change in laboratory values.

The bounds of the boxes indicate the 25th and 75th percentiles; the whiskers indicate the 5th and 95th percentiles. To convert creatinine values to µmol/L, multiply by 88.4. ESR = erythrocyte sedimentation rate.

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Comments

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Immunosuppressive therapy in Idiopathic retroperitoneal fibrosis
Posted on May 10, 2011
Renzo Marcolongo
Padua University, Clinical Immunology Branch
Conflict of Interest: None Declared

Dear Sir, we read with interest the paper by Paul J. Scheel Jr. et al. describing the outcome of combined prednisone and mycophenolate mofetil (MMF) treatment in a case series of subjects with Idiopathic Retroperitoneal Fibrosis (IRF) (1). The authors suggest that the combination prednisone and MMF is a potentially effective treatment for IRF. We agree with them that alternative immunosuppressive therapy is required in IRF patients who do not respond to standard therapy. We also agree that their preliminary results are sufficiently promising to warrant properly performed trials, and that therapeutic efficacy of MMF in maintaining remission and in sparing steroids should be assessed. Nevertheless, until then, MMF should be considered to have no clear indications as first line therapy in IRF, since other less aggressive therapeutic approaches may be equally effective. Indeed, as other fibrosing conditions (2, 3), IRF may show a good response to corticosteroid treatment, alone (4,5) or in combination with tamoxifen (4,6), even if the recurrence of the disease has been reported after its discontinuation (7). In addition, we published a case series which included 15 patients receiving a combination of prednisone and azathioprine for at least for three months (8), that proved to be effective and safer than other immunosuppressive combinations. Anyway, we think that MMF and other immunosuppressive agents, for their potential side effects, should be reserved to patients who show resistance to non-immunosuppressive therapeutic regimens, in particular, when, as many IRF patients, they are over 60 years and their metabolic condition is unbalanced or their organ function becomes particularly fragile.

References

1. Scheel PJ Jr, Feeley N, Sozio SM. Combined prednisone and mycophenolate mofetil treatment for retroperitoneal fibrosis: a case series. Ann Intern Med. 2011 Jan 4;154(1):31-6

2. van Bommel EF, Siemes C, Hak LE, et al. Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone. Am J Kidney Dis 2007;49(5):615-25.

3. Kardar AH, Kattan S, Lindstedt E, et al. Steroid therapy for idiopathic retroperitoneal fibrosis: dose and duration. J Urol 2002;168(2):550-5.

4. Moroni G Gallelli B, Banfi G, et al. Long-term outcome of idiopathic retroperitoneal fibrosis treated with surgical and/or medical approaches. Nephrol Dial Transplant 2006.

5. Moody T, Vaughn E: Steroids in the treatment of retroperitoneal fibrosis. J Urol 121:109-111, 1979.

6. Harris C, Goldstein D.Tamoxifen use in retroperitoneal fibrosis. Intern Med J. 2010 Dec;40(12):e5-7.

7. Swartz RD. Idiopathic retroperitoneal fibrosis: a review of the pathogenesis and approaches to treatment. Am J Kidney Dis. 2009 Sep;54(3):546-53. Epub 2009 Jun 10. Review.

8. Marcolongo R, Tavolini IM, Laveder F, et al. Immunosuppressive therapy for idiopathic retroperitoneal fibrosis: a retrospective analysis of 26 cases. Am J Med 2004; 116: 194-97.

Conflict of Interest:

None declared

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