Abstract
Cryopyrin-associated periodic syndrome (CAPS) is an inherited disease which is caused by gain-of function mutations in CIAS1 gene function resulting in increased secretion of active IL-1β. IL-1β also can stimulate the production of other proinflammatory cytokines, such as IL-6, TNFα and so on (downstream cascade). The use of IL-1 blockers dramatically controls inflammation in CAPS patients and provide a persistent amelioration of the inflammatory manifestations. The data about using of IL-6 or TNFα blockers in CAPS are very limited.
Highlights
Cryopyrin-associated periodic syndrome (CAPS) is an inherited disease which is caused by gain-of function mutations in CIAS1 gene function resulting in increased secretion of active IL-1b
The aim of our retrospective study was to describe the effects of IL-6 and TNFa blockers in CINCA/NOMID syndrome
We describe the effects of IL-6 and TNFa blockers in 4 patients with CINCA/NOMID syndrome who were treated in their local hospitals by tocilizumab (n=2), infliximab (n=1) and adalimumab (n=1)
Summary
Cryopyrin-associated periodic syndrome (CAPS) is an inherited disease which is caused by gain-of function mutations in CIAS1 gene function resulting in increased secretion of active IL-1b. IL-1b can stimulate the production of other proinflammatory cytokines, such as IL-6, TNFa and so on (downstream cascade). The use of IL-1 blockers dramatically controls inflammation in CAPS patients and provide a persistent amelioration of the inflammatory manifestations. The data about using of IL-6 or TNFa blockers in CAPS are very limited
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