Sandra Hansmann

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OBJECTIVES Muckle-Wells syndrome (MWS) is an autoinflammatory disease characterized by excessive interleukin-1 (IL-1) release, resulting in recurrent fevers, sensorineural hearing loss, and amyloidosis. IL-1 inhibition with anakinra, an IL-1 receptor antagonist, improves clinical symptoms and inflammatory markers. Subclinical disease activity is commonly(More)
BACKGROUND Muckle-Wells-syndrome (MWS) is an autoinflammatory disease characterized by systemic and organ-specific inflammation due to excessive interleukin (IL)-1 release. Inner ear inflammation results in irreversible sensorineural hearing loss, if untreated. Early recognition and therapy may prevent deafness. The aims of the study were to characterize(More)
BACKGROUND Juvenile idiopathic arthritis (JIA) is a chronic illness with a high risk of developing long-term disability. Disease activity is currently being monitored and quantified by ACR core set. Here, joint inflammation is determined; however joint function is the crucial component for developing disability. The aim of this study was to quantify and(More)
Purpose Muckle-Wells syndrome (MWS) is a rare inherited auto-inflammatory disease. Patients may present with fever, rash, arthralgia, or conjunctivitis. Effective treatment of MWS has become available with the advance of IL-1 inhibition preventing disease sequalae of sensorineural deafness and amyloidosis. Therefore, early diagnosis of MWS is crucial to(More)