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Rheumatology Advance Access originally published online on April 3, 2008
Rheumatology 2008 47(7):946-951; doi:10.1093/rheumatology/ken118
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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

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Autoinflammatory diseases: an update of clinical and genetic aspects

Q. Yao and D. E. Furst

Division of Rheumatology, David Geffen Medical School at UCLA, Los Angeles, CA, USA.

Correspondence to: D. E. Furst, Division of Rheumatology, UCLA, 1000 Veteran Ave., Rehab 39-52, Los Angeles, CA 90095, USA. E-mail: defurst{at}mednet.ucla.edu


  Abstract

To review clinical manifestations and genetic features of the autoinflammatory diseases, a group of rare, genetically defined diseases which have been newly grouped into a coherent whole. We performed a literature review using the keywords ‘periodic fever syndrome’, ‘autoinflammatory disease’ and ‘therapy’. All relevant original and review articles in English were reviewed. A case report of each autoinflammatory disease was excerpted from the literature and presented. This review summarizes the clinical manifestations, genetic analysis and therapy of FMF, TNF-{alpha} receptor-associated periodic fever syndrome, hyperimmunoglobulinaemia D periodic fever syndrome and cryopyrin-associated periodic fever syndrome. These diseases have periodic fever, are hereditary and recurrent, with elevated acute-phase reactants. Differentiating features of these disorders are tabulated. Autoinflammatory diseases have some communalities in their presentation although they represent a relatively diverse group of genetically associated diseases.

KEY WORDS: Autoinflammatory disease, Familial Mediterranean fever, Tumour necrosis factor a receptor associated periodic fever syndrome, Hyperimmunoglobulinemia D periodic fever syndrome, Cryopyrin, Genetic analysis, Amyloidosis, Therapy, TNF{alpha} blocker, Interleukin-1 receptor antagonist

Submitted 24 January 2008; Accepted 18 February 2008


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