Canakinumab as effective therapy in anakinra-refractory Schnitzler syndrome: a case-based review.

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.