• Media type: E-Article
  • Title: Cryopyrin‐associated periodic syndrome in early childhood can be successfully treated with interleukin‐1 blockades
  • Contributor: Eskola, Vesa; Pohjankoski, Heini; Kröger, Liisa; Aalto, Kristiina; Latva, Katariina; Korppi, Matti
  • imprint: Wiley, 2018
  • Published in: Acta Paediatrica
  • Language: English
  • DOI: 10.1111/apa.14217
  • ISSN: 0803-5253; 1651-2227
  • Keywords: General Medicine ; Pediatrics, Perinatology and Child Health
  • Origination:
  • Footnote:
  • Description: <jats:title>Abstract</jats:title><jats:sec><jats:label /><jats:p>Cryopyrin‐associated periodic syndrome (<jats:styled-content style="fixed-case">CAPS</jats:styled-content>) is caused by a mutation in the <jats:italic><jats:styled-content style="fixed-case">NLRP</jats:styled-content>3</jats:italic> gene encoding cryopyrin production. Overproduction of interleukin‐1 (<jats:styled-content style="fixed-case">IL</jats:styled-content>‐1) leads to symptoms that are associated with elevated inflammatory markers, including periodic fever and a rash. We provide a clinical overview of <jats:styled-content style="fixed-case">CAPS</jats:styled-content> in children, including three Finnish case studies.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>When <jats:styled-content style="fixed-case">CAPS</jats:styled-content> has been diagnosed, an <jats:styled-content style="fixed-case">IL</jats:styled-content>‐1 blockade with biological should be introduced to lessen the symptoms and to prevent the progression of organ damage.</jats:p></jats:sec>