The IL-1 receptor antagonist anakinra (kineret®) stabilizes the NLRP3 mutation-specific risk for hearing loss in patients with severe cryopyrin-associated periodic syndromes (CAPS)

Introduction CAPS is a rare monogenic autoinflammatory syndrome consisting of a spectrum of three conditions: Familial cold autoinflammatory syndrome (FCAS), MuckleWells syndrome (MWS), and the most severe form, NOMID/CINCA. Progressive hearing loss is a characteristic of severe CAPS [1]. Previous analyses showed that long-term anakinra treatment stabilized the progression of hearing loss [2,3]. CAPS patients with different NLRP3 mutations have distinctly different trajectories of hearing loss, suggesting a mutation-specific risk that should be considered when making treatment decisions [4].