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Review
. 2010 Apr;12(2):101-7.
doi: 10.1007/s11926-010-0086-1.

Hyperimmunoglobulin D syndrome in childhood

Jeroen C H van der Hilst  1 , Joost Frenkel
Affiliations
Review

Hyperimmunoglobulin D syndrome in childhood

Jeroen C H van der Hilst et al. Curr Rheumatol Rep. 2010 Apr.

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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