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Rheumatology Advance Access published online on August 7, 2008

Rheumatology, doi:10.1093/rheumatology/ken291
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© The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Successful mycophenolate mofetil therapy in nine patients with idiopathic retroperitoneal fibrosis

S. Adler1,2, S. Lodermeyer1, J. Gaa3 and U. Heemann1

1Department of Internal Medicine II/Nephrology, Technical University of Munich, Munich, Germany 2Department of Rheumatology and Clinical Immunology/Allergology, Inselspital, University of Bern, Bern, Switzerland and 3Department of Radiology, Technical University of Munich, Munich, Germany.

Correspondence to: S. Adler, Department of Rheumatology and Clinical Immunology/Allergology, Inselspital, University of Bern, Freiburgstrasse 3010, Bern, Switzerland. E-mail: sabine.adler{at}lrz.tu-muenchen.de


   Abstract

Objective. To assess the therapeutic benefit of mycophenolate mofetil (MMF) in retroperitoneal fibrosis (RF).

Methods. MMF 2 g/day and prednisone 1 mg/kg were initiated in nine patients with radiological (9/9) and histological verification (2/9) of idiopathic RF. Out of nine patients, seven needed bilateral ureteral stenting due to extensive hydronephrosis.

Results. All patients experienced regression of radiological extension. Out of seven patients, five were free of ureteral catheters after a mean of 5.6 months and two remained on stenting due to secondary stenosis. Within 6 months mean creatinine and CRP fell from 2.5 to 1.2 mg/dl and from 4.0 to 1.4 mg/dl, respectively. MMF was discontinued after a mean of 27 months. Prednisone was tapered to zero after a mean of 7 months. Side-effects were urinary tract infections in 7/9 patients and impaired glucose tolerance in 3/9. No recurrence occurred after withdrawal of glucocorticoids and MMF in 7/9 patients after a mean overall follow-up of 55 months (range 12–120).

Conclusions. Treatment with MMF and glucocorticoids was successful in inducing partial or complete and lasting remission in RF. The results suggest the use of MMF as additional immunosuppressive option.

KEY WORDS: Mycophenolate mofetil, Retroperitoneal fibrosis, Glucocorticoids, Connective tissue disease

Submitted 13 April 2008; revised version accepted 25 June 2008.
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