Periodic fever syndrome

Periodic fever syndrome

The periodic fever syndromes (also known as autoinflammatory syndromes) are a set of disorders, many of which are genetic disorders in which the mechanisms which initiate and control inflammation are disturbed, leading to uncontrolled inflammation throughout the body. The syndromes are diverse, but tend to cause fever, joint pains, abdominal pains and may lead to chronic complications such as amyloidosis.cite journal |author=Stojanov S, Kastner DL |title=Familial autoinflammatory diseases: genetics, pathogenesis and treatment |journal=Curr Opin Rheumatol |volume=17 |issue=5 |pages=586–99 |year=2005 |pmid=16093838 |doi=]

Periodic fever syndromes are medically distinct from immunodeficiency conditions, in which infections can occur frequently and induce fever.

Individual periodic fever syndromes

*Familial Mediterranean fever (FMF)
*Hyperimmunoglobulinemia D with recurrent fever (HIDS). This is now (along with mevalonic aciduria) defined as a mevalonate kinase deficiencycite journal |author=Houten SM, Frenkel J, Waterham HR |title=Isoprenoid biosynthesis in hereditary periodic fever syndromes and inflammation |journal=Cell. Mol. Life Sci. |volume=60 |issue=6 |pages=1118–34 |year=2003 |pmid=12861380 |doi=10.1007/s00018-003-2296-4]
*TNF receptor associated periodic syndrome (TRAPS)
*Muckle-Wells syndrome
*Familial cold urticaria
*Neonatal onset multisystem inflammatory disease
*Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA syndrome or Marshall syndrome (not Marshall-Smith syndrome))
*Blau syndrome
*Pyogenic sterile arthritis, pyoderma gangrenosum, acne (PAPA)

References


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  • Periodic fever, aphthous stomatitis, pharyngitis and adenitis — (PFAPA) syndrome is a medical condition, typically starting in young children, in which high fever occurs periodically at intervals of about 3 5 weeks, frequently accompanied by aphthous ulcers, pharyngitis and/or adenitis. The syndrome was… …   Wikipedia

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  • Syndrome de fievre periodique — Syndrome de fièvre périodique Le syndrome de fièvre périodique comprends plusieurs maladies, génétiques ou infectieuses, caractérisées par des épisodes récurrents de syndromes inflammatoires comprenant fièvre, arthralgies ... Sommaire 1 Maladies… …   Wikipédia en Français

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  • Hyperimmunoglobulinemia D with recurrent fever — (commonly abbreviated as HIDS) is a periodic fever syndrome originally described in 1984 by the internist Prof. Jos van der Meer, then at Leiden University Medical Centre. No more than 300 cases have been described worldwide.FeaturesHIDS is one… …   Wikipedia

  • Marshall-Smith syndrome — Discovered in 1971 (Marshall, Graham, Scott, Boner, Smith). Marshall Smith Syndrome is characterized by unusual accelerated skeletal maturation (usually starting before birth) and symptoms like conspicuous physical characteristics, respiratory… …   Wikipedia

  • TNF receptor associated periodic syndrome — (also known as TRAPS or familial Hibernian fever) is a periodic fever syndrome associated with mutations in a receptor for the molecule tumor necrosis factor (TNF) that is inheritable in an autosomal dominant manner. Individuals with TRAPS have… …   Wikipedia

  • Marshall–Smith syndrome — Marshall Smith syndrome Classification and external resources ICD 10 GroupMajor.minor ICD 9 xxx …   Wikipedia

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