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

页码：577 / 580

页数：4

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