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Cryopyrin‐associated periodic syndrome in early childhood can be successfully treated with interleukin‐1 blockades
Author(s) -
Eskola Vesa,
Pohjankoski Heini,
Kröger Liisa,
Aalto Kristiina,
Latva Katariina,
Korppi Matti
Publication year - 2018
Publication title -
acta paediatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/apa.14217
Subject(s) - medicine , rash , blockade , interleukin , overproduction , canakinumab , immunology , interleukin 6 , interleukin 1β , mutation , inflammation , gene , cytokine , anakinra , genetics , disease , receptor , biology
Cryopyrin‐associated periodic syndrome ( CAPS ) is caused by a mutation in the NLRP 3 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.