What is it about?

Cryopyrin-associated periodic syndromes (CAPS) are a subgroup of the hereditary periodic fever syndromes, which are rare autoinflammatory and inherited disorders, characterized by recurrent inflammation. Rilonacept (Arcalyst(TM); Regeneron) is the first us Food and Drug Administration-approved treatment for familial cold autoinflammatory syndrome and Muckle-Wells syndrome and the first in a new line of drugs designed for longer-acting IL-1 blockade. Rilonacept has been associated with a decrease in disease activity in the treatment of CAPS. The clinical safety and efficacy of rilonacept in CAPS and non-CAPS populations is summarized in this review.

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Why is it important?

Rilonacept is also beneficial for patients who tolerate injections poorly, due to an extended half-life over the unapproved CAPS treatment, anakinra (a shorter acting agent), requiring weekly rather than daily self-administration. Other autoinflammatory disorders may also benefit from rilonacept treatment, with clinical trials in progress for systemic onset juvenile idiopathic arthritis, gout and familial mediterranean fever.


A definite advance with the downside of annual cost: $250,000. Manufacturer: Regeneron

Michael McDermott
University of Leeds

Read the Original

This page is a summary of: Rilonacept in the management of cryopyrin-associated periodic syndromes (CAPS), Journal of Inflammation Research, January 2010, Taylor & Francis, DOI: 10.2147/jir.s8109.
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