Cryopyrin-associated periodic syndrome in early childhood can be successfully treated with interleukin-1 blockades

被引：7
|
作者：
Eskola, Vesa [1 ]
Pohjankoski, Heini [2 ]
Kroeger, Liisa [3 ]
Aalto, Kristiina [4 ]
Latva, Katariina [2 ]
Korppi, Matti [1 ,5 ]
机构：
[1] Tampere Univ Hosp, Dept Pediat, POB 2000, FIN-33521 Tampere, Finland
[2] Pajjat Hame Dist Cent Hosp, Dept Pediat, Lahti, Finland
[3] Kuopio Univ Hosp, Dept Pediat, Kuopio, Finland
[4] Helsinki Univ Hosp, Dept Pediat, Helsinki, Finland
[5] Univ Tampere, Pediat Res Ctr, Tampere, Finland
来源：
ACTA PAEDIATRICA | 2018年 / 107卷 / 04期
关键词：
Biologics; Children; Cryopyrin-associated periodic syndrome; Interleukin-1; Periodic fever; SUSTAINED RESPONSE; OPEN-LABEL; CANAKINUMAB; EFFICACY; SAFETY; MANAGEMENT; RILONACEPT; PHENOTYPE; ANAKINRA; LIFE;
D O I：
10.1111/apa.14217
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
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.
引用
收藏
页码：577 / 580
页数：4
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