Cryopyrin-associated periodic syndrome

Cryopyrin-associated periodic syndrome, abbreviated CAPS, is a group of rare and heterogeneous autoinflammatory diseases marked by systemic inflammation driven by interleukin 1 beta. Its clinical effects involve the skin, joints, central nervous system and eyes. It covers a spectrum of three clinically overlapping syndromes that were once thought to be separate entities: familial cold autoinflammatory syndrome, formerly called familial cold-induced urticaria; Muckle-Wells syndrome; and neonatal-onset multisystem inflammatory disease, also called chronic infantile neurologic cutaneous and articular syndrome. These share a single genetic mutation and a common pathogenic pathway. A fourth form, keratoendotheliitis fugax hereditaria, is included in which the autoinflammatory symptoms affect only the anterior segment of the eye.