Rilonacept in the management of cryopyrin-associated periodic syndromes (CAPS)

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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Photo by Maxim Shklyaev on Unsplash

Photo by Maxim Shklyaev on Unsplash

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.

Perspectives