Department of Pediatrics, Tampere University Hospital, Tampere, Finland.
Department of Pediatrics, Päijät-Häme District Central Hospital, Lahti, Finland.
Acta Paediatr. 2018 Apr;107(4):577-580. doi: 10.1111/apa.14217. Epub 2018 Feb 5.
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.
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.
冷吡啉相关周期性综合征 (CAPS) 是由编码冷吡啉产生的 NLRP3 基因突变引起的。白介素-1 (IL-1) 的过度产生导致与炎症标志物升高相关的症状,包括周期性发热和皮疹。我们提供了儿童 CAPS 的临床概述,包括三个芬兰病例研究。
一旦诊断出 CAPS,应采用生物制剂阻断 IL-1 以减轻症状并防止器官损伤的进展。