Article
Cryopyrin-associated periodic syndrome.
The Israel Medical Association journal : IMAJ - 1 Oct 2014
Giat Eitan, Lidar Merav
Abstract excerpt
CAPS is a rare autoinflammatory disease associated with mutations in the NLRP3 gene that result in overactivation of the inflammasome, increased secretion of IL-1beta and IL-18, and systemic inflammation. Genetic testing has allowed for grouping of the three, previously distinct clinical syndromes of FCAS, MWS and NOMID, into a single syndrome termed CAPS. The clinical features include urticarial rash and fever,...
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