Journal
RHEUMATOLOGY
Volume 47, Issue 7, Pages 946-951Publisher
OXFORD UNIV PRESS
DOI: 10.1093/rheumatology/ken118
Keywords
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
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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.
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