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Current Rheumatology Reports
Published in: Current Rheumatology Reports 2/2010

01-04-2010

Hyperimmunoglobulin D Syndrome in Childhood

Authors: Jeroen C. H. van der Hilst, Joost Frenkel

Published in: Current Rheumatology Reports | Issue 2/2010

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Abstract

Hyperimmunoglobulinemia D and periodic fever syndrome, an autoinflammatory syndrome, is caused by mutations in the gene coding for mevalonate kinase. The disease is clinically characterized by recurrent attacks of fever accompanied by an array of inflammatory symptoms including lymphadenopathy, rash, arthritis, and gastrointestinal complaints. Most patients have their first attack in the first year of life, typically after a childhood vaccination. The frequency of attacks is highest during childhood, with a gradual decrease after adolescence. Frequent fever attacks impair quality of life and the achievement of educational milestones. Recent reports show promising results with anakinra and etanercept to treat the attacks.
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Metadata
Title
Hyperimmunoglobulin D Syndrome in Childhood
Authors
Jeroen C. H. van der Hilst
Joost Frenkel
Publication date
01-04-2010
Publisher
Current Science Inc.
Published in
Current Rheumatology Reports / Issue 2/2010
Print ISSN: 1523-3774
Electronic ISSN: 1534-6307
DOI
https://doi.org/10.1007/s11926-010-0086-1

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