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

Introduction: Schnitzler's syndrome (SchS) is a disabling autoinflammatory disorder, characterized by 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.

TLR2/TLR4-dependent exaggerated cytokine production in hyperimmunoglobulinaemia D and periodic fever syndrome.

Objective: The autoinflammatory hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS) is characterized by recurrent episodes of fever and inflammation. As part of the mevalonate kinase deficiency spectrum, it is caused by MVK mutations, resulting in decreased mevalonate kinase activity in the isoprenoid pathway. Although IL-1β is considered a major cytokine in its pathogenesis, IL-1 blockade is not successful in a proportion of patients.