RESUMO
Autoinflammatory diseases (AID) in childhood is one of refractory diseases, whose pathogenesis is not completely clear. In recent years, a large number of studies have shown that NLRP3 inflammasome plays an important role in the development of AIDs in children. Inflammasome is a cytosolic multiprotein complex that can activate cysteinyl aspartate-specific protease-1 (caspase-1), to further promote the maturation and secretion of proinflammatory cytokines IL-1β and IL-18 as well as pyroptosis and regulate innate immune response. IL-1 receptor antagonist (Anakinra) and IL-1β monoclonal antibody (Canakinumab) have good therapeutic effects in children with AIDs. This article reviews the research progress of NLRP3 inflammasome in the pathogenesis of autoinflammatory diseases.
RESUMO
Juvenile idiopathic arthritis (JIA)is one of the most common chronic rheumatic diseases in childhood.Systemic juvenile idiopathic arthritis(SJIA) is a subtype of JIA,which may be different from other subtypes.As shown in studies,interleukin-6 (IL-6),IL-18,IFN-γ,may instead of TNF-α,play an important role in SJIA.Traditional biological agents such as TNF antagonists have limited effect in the patients with SJIA.Tocilizumab(TCZ),a humanized anti-IL-6 receptor monoclonal antibody,inhibits the binding of IL-6 with transmembrane IL-6R or soluble IL-6R,blocking the IL-6 mediated inflammation and joint destruction.This article reviews the efficacy and safety of TCZ in patients with SJIA.