Which interleukin is produced in excess in cryopyrinopathies?

In cryopyrinopathies, a group of autoinflammatory diseases, there is an excessive production of interleukin-1 beta (IL-1β). These conditions are associated with mutations in the NLRP3 gene, which plays a crucial role in the formation of the inflammasome, a complex that activates caspase-1. This activation leads to the conversion of pro-inflammatory cytokines into their active forms, particularly IL-1β.

IL-1β is a potent pro-inflammatory cytokine that contributes to the inflammatory processes seen in these disorders. Elevated levels of IL-1β can lead to features such as recurrent fever, rash, and joint pain, which are hallmark symptoms of cryopyrinopathies like Familial Cold Autoinflammatory Syndrome and Muckle-Wells Syndrome.

Understanding the role of IL-1β in these diseases has significant clinical implications, particularly in the development of targeted therapies. Medications such as anakinra, an IL-1 receptor antagonist, have shown effectiveness in managing symptoms by blocking the action of IL-1β. This highlights the importance of IL-1β as the key cytokine involved in the pathophysiology of cryopyrinopathies.