Journal
BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY
Volume 28, Issue 2, Pages 263-276Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.berh.2014.05.005
Keywords
Periodic fever; Autoinflammatory diseases; PFAPA syndrome; Familiar Mediterranean fever; Cryopyrin-associated periodic syndromes
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Autoinflammatory diseases are characterized by the presence of chronic or recurrent systemic inflammation secondary to abnormal activation of innate immunity pathways. Many of these diseases have been found to have mutations in the genes within these pathways. Due to their rarity, non-specific symptoms and the very recent genetic and phenotypic identification and recognition, a delay in diagnosis is common. Nevertheless, some specific clinical features should help the clinician to make the diagnosis. The purpose of this article is to provide a brief clinical description of these conditions and to present clinical flow-charts useful for a correct diagnosis of children with suspected autoinflammatory syndromes. (C) 2014 Elsevier Ltd. All rights reserved.
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