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.
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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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