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Periodic fever syndromes

Lachmann, HJ; (2017) Periodic fever syndromes. Best Practice & Research Clinical Rheumatology , 31 (4) pp. 596-609. 10.1016/j.berh.2017.12.001. Green open access

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Abstract

Periodic fever syndromes are autoinflammatory diseases. The majority present in infancy or childhood and are characterised by recurrent episodes of fever and systemic inflammation that occur in the absence of autoantibody production or identifiable infection. The best recognised disorders include CAPS, FMF, TRAPS and MKD. Understanding the molecular pathogenesis of these disorders provides unique insights into the regulation of innate immunity. Diagnosis relies on clinical acumen and is supported by genetic testing. With the exception of FMF, which is prevalent in populations originating from the Mediterranean, these syndromes are rare and easily overlooked in the investigation of recurrent fevers. Disease severity varies from mild to life threatening, and one of the most feared complications is AA amyloidosis. Effective therapies are available for many of the syndromes, including colchicine, IL-1 blockade and anti-TNF therapies, and there is an increasing interest in blocking interferon pathways.

Type: Article
Title: Periodic fever syndromes
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.berh.2017.12.001
Publisher version: http://dx.doi.org/10.1016/j.berh.2017.12.001
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Cryopyrin-associated periodic syndromes (CAPS), Familial Mediterranean fever (FMF), Tumour necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS), Mevalonate kinase deficiency (MKD), Deficiency of IL-1 receptor antagonist (DIRA), Deficiency of IL-36 receptor antagonist (DITRA), Blau syndrome, Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome (CANDLE), Deficiency of adenosine deaminase 2 (DADA2), Aphthous stomatitis, Pharyngitis and cervical adenitis (PFAPA)
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: https://discovery.ucl.ac.uk/id/eprint/10053943
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