アナキンラ抵抗性のシュニッツラー症候群に対するカナキヌマブの効果的な治療法:症例に基づくレビュー
Sergio De Santos Belinchón1, Cristina Ausín García1,2, Pablo Daniel Lorenzo Barcia3
1Department of Internal Medicine, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
Abstract:
Schnitzler syndrome is a rare autoinflammatory syndrome caused by a dysregulation of the interleukin-1β signalling pathway. Symptoms tend to appear in middle age and include urticariform skin lesions, fever, bone pain, lymphadenopathy and IgM monoclonal gammopathy. Interleukin-1 (IL-1) targeted therapy constitutes the basis for treatment and can lead to complete resolution of symptoms. We report a case of Schnitzler syndrome in a 49-year-old man refractory to anakinra and subsequently treated successfully with canakinumab.
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