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Muckle-Wells syndrome (MWS), a phenotype within the spectrum of cryopyrine-associated periodic syndrome (CAPS) is characterized by excessive IL-1 release resulting in chronic systemic and organ-specific inflammation including sensorineural hearing loss. During continuous anti-IL-1-therapy clinical symptoms are controlled and hearing loss remains stable. Limited data exists about discontinuation of IL-1-inhibition during the course of disease.

IL-1 inhibition in Muckle-Wells-Syndrome: withdrawal resulting in rapid deterioration of hearing loss