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NDT Advance Access originally published online on November 25, 2008
Nephrology Dialysis Transplantation 2009 24(2):676-678; doi:10.1093/ndt/gfn646
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© The Author [2008]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org


Successful treatment of familial Mediterranean fever with Anakinra and outcome after renal transplantation

Christian Moser1, Gudrun Pohl2, Isabella Haslinger3, Sylvia Knapp3, Dorota Rowczenio4, Tonia Russel4, Helen J. Lachmann4, Ursula Lang1 and Josef Kovarik1

1 6th Medical Department with Nephrology and Dialysis 2 1st Medical Department with Oncology, Wilhelminenspital 3 Centre for Molecular Medicine of the Austrian Academy of Sciences, Department of Medicine 1, Medical University of Vienna, Austria 4 Department of Medicine, National Amyloidosis Centre, Royal Free and University College Medical School, Hampstead Campus, London, UK

Correspondence and offprint requests to: Christian Moser, 6th Medical Department with Nephrology and Dialysis, Wilhelminenspital, 1160 Vienna, Montleartstrasse 37, Austria. Tel: +43-6504715330; Fax: +43-1-49150-2609; E-mail: Christian.moser.md{at}aon.at



  Abstract

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent episodes of fever and inflammation. The most severe complication of FMF is the development of AA amyloidosis, which can be life threatening. The only current effective treatment for FMF is colchicine. Regular prophylactic treatment with colchicine at a dose of 1–2 mg daily prevents or substantially reduces the clinical manifestations of FMF in at least 90% of cases. However, ~10% of patients are reported to be resistant or non-responsive to colchicine and in these cases there is no consensus as to which second line agents should be used. We describe the first case, to our knowledge, of a patient with FMF and end-stage renal failure due to AA amyloidosis, successfully treated with IL-1 receptor blockade. Our data suggest that the IL-1 receptor antagonist Anakinra (Kineret®; r-metHuIL-1 ra) may represent a safe and effective therapy for the treatment of colchicine-resistant FMF, in patients requiring renal replacement therapy, with dialysis or transplantation.

Keywords: Anakinra; colchicine; familial Mediterranean fever; interleukin-1 receptor antagonist

Received for publication: 15.10.08
Accepted in revised form: 27.10.08


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