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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. <\/jats:p>","DOI":"10.12968\/bjon.2019.28.18.1180","type":"journal-article","created":{"date-parts":[[2019,10,9]],"date-time":"2019-10-09T21:56:11Z","timestamp":1570658171000},"page":"1180-1186","source":"Crossref","is-referenced-by-count":4,"title":["Recognising and understanding cryopyrin-associated periodic syndrome in adults"],"prefix":"10.12968","volume":"28","author":[{"given":"Ren\u00e9","family":"Williams","sequence":"first","affiliation":[{"name":"at time of writing was CAPS Clinical Nurse Specialist, National Amyloidosis Centre, Division of Medicine, University College London"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philip","family":"Hawkins","sequence":"additional","affiliation":[{"name":"Professor of Medicine, National Amyloidosis Centre, Division of Medicine, University College London"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thirusha","family":"Lane","sequence":"additional","affiliation":[{"name":"Honorary Research Associate, National Amyloidosis Centre, Division of Medicine, University College London"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"5130","reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1002\/art.10509"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2010.138420"},{"key":"B4","first-page":"433","volume":"66","author":"De Boeck H","year":"2000","journal-title":"Acta Orthopaedica Belgica"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V87.6.2095.bloodjournal8762095"},{"key":"B6","first-page":"1","volume":"3","author":"Gillespie J","year":"2010","journal-title":"J Inflamm Res"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa055137"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200306193482523"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1002\/art.23687"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-med-061512-150641"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1186\/ar3535"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1177\/1759720X13502629"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2011.152728"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1186\/ar2579"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20082481"},{"key":"B16","unstructured":"Lane T. 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