Journal
ACTA PAEDIATRICA
Volume 107, Issue 4, Pages 577-580Publisher
WILEY
DOI: 10.1111/apa.14217
Keywords
Biologics; Children; Cryopyrin-associated periodic syndrome; Interleukin-1; Periodic fever
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Cryopyrin-associated periodic syndrome (CAPS) is caused by a mutation in the NLRP3 gene encoding cryopyrin production. Overproduction of interleukin-1 (IL-1) leads to symptoms that are associated with elevated inflammatory markers, including periodic fever and a rash. We provide a clinical overview of CAPS in children, including three Finnish case studies. Conclusion: When CAPS has been diagnosed, an IL-1 blockade with biological should be introduced to lessen the symptoms and to prevent the progression of organ damage.
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