Monogenic autoinflammatory disorders: beyond the periodic fever

The past two decades have seen an exponential increase in the number of monogenic autoinflammatory disorders described, coinciding with improved genetic sequencing techniques. This group of disorders has evolved to be heterogeneous and certainly more complex than the original four ‘periodic fever sy...

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Bibliographic Details
Published inInternal medicine journal Vol. 50; no. 2; pp. 151 - 164
Main Author Moghaddas, Fiona
Format Journal Article
LanguageEnglish
Published Melbourne John Wiley & Sons Australia, Ltd 01.02.2020
Wiley Subscription Services, Inc
Subjects
autoimmunity
Autoimmunity - immunology
autoinflammation
autoinflammatory disease
Cytokines - immunology
Fever
Hereditary Autoinflammatory Diseases - genetics
Hereditary Autoinflammatory Diseases - immunology
Humans
immune deficiency
immune dysregulation
Immunity, Innate - immunology
Immunologic Deficiency Syndromes - immunology
Inflammation - immunology
periodic fever syndrome
autoinflammation
immune deficiency
autoimmunity
periodic fever syndrome
autoinflammatory disease
immune dysregulation
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Summary:The past two decades have seen an exponential increase in the number of monogenic autoinflammatory disorders described, coinciding with improved genetic sequencing techniques. This group of disorders has evolved to be heterogeneous and certainly more complex than the original four ‘periodic fever syndromes’ caused by innate immune over‐activation. This review aims to provide an update on the classic periodic fever syndromes as well as introducing the broadening spectrum of clinical features seen in more recently described conditions.
Bibliography:ObjectType-Article-2
SourceType-Scholarly Journals-1
ObjectType-Feature-3
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ObjectType-Review-1
ISSN:1444-0903
1445-5994
DOI:10.1111/imj.14414