The role of interleukin-1 beta in the pathophysiology of Schnitzler’s syndrome

Schnitzler’s syndrome (SchS) is a disabling autoinflammatory disorder, characterized by chronic urticaria a chronic urticarial rash, an M-protein, arthralgia, and other signs of systemic inflammation. Anti-interleukin-1 (IL-1) beta antibodies are highly effective, but the pathophysiology is still largely unknown. Here we studied the effect of in-vivo IL-1 inhibition on serum markers of inflammation and cellular immune responses. Transcriptome analysis of RNA from PBMCs from three patients with Schnitzler’s syndrome and three matched controls. Patient samples were drawn during symptomatic disease, anakinra and canakinumab treatment.