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Recognising and understanding cryopyrin-associated periodic syndrome in adults

Williams, R; Hawkins, P; Lane, T; (2019) Recognising and understanding cryopyrin-associated periodic syndrome in adults. British Journal of Nursing , 28 (18) pp. 1180-1186. 10.12968/bjon.2019.28.18.1180. Green open access

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Abstract

Cryopyrin-associated periodic syndrome (CAPS) is a group of rare hereditary autoinflammatory diseases characterised by recurrent flares of mild to severe systemic inflammation and fever. CAPS is the umbrella term for a spectrum of individual conditions, namely familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID), also known as chronic infantile neurologic, cutaneous and articular (CINCA) syndrome. The flare symptoms include fever, fatigue, rashes, headaches, arthralgia and myalgia that can last for a few hours or for several days. These symptoms are debilitating, contributing to poor quality of life for patients if left untreated. Serious life-changing complications such as hearing loss, blindness and AA amyloidosis resulting in kidney failure can occur. Until recently, treatment of the disease was symptomatic using non-steroidal anti-inflammatory and immunosuppressant drugs with limited success. In contrast, biological treatments targeting interleukin 1 (IL-1) have proved remarkably effective, often associated with complete and sustained disease remission, vastly improved quality of life and avoidance of serious long-term complications.

Type: Article
Title: Recognising and understanding cryopyrin-associated periodic syndrome in adults
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.12968/bjon.2019.28.18.1180
Publisher version: https://doi.org/10.12968/bjon.2019.28.18.1180
Language: English
Additional information: © 2019 The Author(s). This is an Open Access article published by MA Healthcare Ltd and distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license (https://creativecommons.org/licenses/by-nc/4.0/).
Keywords: Autoinflammatory disease, Cryopyrin-associated periodic syndrome, Interleukin 1, Biological treatment
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/10092525
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