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Vol. 223, No. 2, 2011
Issue release date: November 2011
Section title: Case and Review
Dermatology 2011;223:113–118
(DOI:10.1159/000331580)

Muckle-Wells Syndrome Effectively Treated with Canakinumab: Is the Recommended Dosing Schedule Mandatory?

Mueller S.M. · Itin P. · Haeusermann P.
Department of Dermatology, University Hospital Basel, Basel, Switzerland

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Article / Publication Details

First-Page Preview
Abstract of Case and Review

Received: 6/16/2011 11:12:55 AM
Accepted: 7/10/2011
Published online: 9/29/2011

Number of Print Pages: 6
Number of Figures: 3
Number of Tables: 0

ISSN: 1018-8665 (Print)
eISSN: 1421-9832 (Online)

For additional information: http://www.karger.com/DRM

Abstract

Cryopyrin-associated periodic fever syndrome (CAPS) is a rare inherited disease due to mutations in the NLRP3 (also called CIAS1) gene on chromosome 1q44 resulting in overproduction of interleukin-1. CAPS comprises three clinically overlapping disorders including Muckle-Wells syndrome. We report on two half-siblings with Muckle-Wells syndrome who were successfully treated with the interleukin-1 beta antibody canakinumab. Despite reduced dosing and longer treatment intervals compared to the recommended dosing schedule (e.g. 150 mg every 8 weeks), the efficacy and tolerability of canakinumab was impressive in both patients. The pharmacologic properties of canakinumab are reviewed and the clinical and economical aspects highlighted. We show that with individualized ‘reflare-guided’ administrations of canakinumab overall costs could hypothetically be reduced by 50% (approx. USD 60,000/patient/year) and therefore could have a major impact on treatment costs.


  

Author Contacts

Simon M. Mueller
Department of Dermatology, University Hospital Basel
Petersgraben 4
CH–4031 Basel (Switzerland)
Tel. +41 61 265 4080, E-Mail muellersim@uhbs.ch

  

Article Information

Received: June 16, 2011
Accepted after revision: July 10, 2011
Published online: September 29, 2011
Number of Print Pages : 6
Number of Figures : 3, Number of Tables : 0, Number of References : 24

  

Publication Details

Dermatology

Vol. 223, No. 2, Year 2011 (Cover Date: November 2011)

Journal Editor: Saurat J.-H. (Geneva)
ISSN: 1018-8665 (Print), eISSN: 1421-9832 (Online)

For additional information: http://www.karger.com/DRM


Article / Publication Details

First-Page Preview
Abstract of Case and Review

Received: 6/16/2011 11:12:55 AM
Accepted: 7/10/2011
Published online: 9/29/2011

Number of Print Pages: 6
Number of Figures: 3
Number of Tables: 0

ISSN: 1018-8665 (Print)
eISSN: 1421-9832 (Online)

For additional information: http://www.karger.com/DRM


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Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
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