Combined interleukin-1 blockade for central nervous system inflammation in children with severe cryopyrin-associated periodic syndromes

Abstract

Cryopyrin-associated periodic syndromes (CAPS) are rare NLRP3-mediated autoinflammatory disorders driven by excessive interleukin-1 (IL-1) signalling [1]. Central nervous system (CNS) inflammation, including aseptic meningitis, papilledema and sensorineural hearing loss, characterises severe CAPS and can cause irreversible damage [2]. Although both anakinra and canakinumab inhibit IL-1, anakinra is often preferred for CNS disease because of superior blood-brain barrier penetration; cerebrospinal fluid (CSF) inflammation may persist under canakinumab [3,4].