Autoinflammatory syndrome(AIS)is characterized by periodic inflammation in the absence of infection,autoantibodies or self-reactive lymphocytes.Therefore,AIS has defining characteristics that differ from those of auto...Autoinflammatory syndrome(AIS)is characterized by periodic inflammation in the absence of infection,autoantibodies or self-reactive lymphocytes.Therefore,AIS has defining characteristics that differ from those of autoimmune diseases.The sterile inflammation observed in AIS is likely caused by the dysregulation of the innate immune system.However,the etiologies and physiopathologies of these autoinflammatory disorders remain ambiguous.Uncontrolled interleukin(IL)-1 biosynthesis,which is initiated by danger signals in the inflammasome or via nucleic acid-sensing pathways,has been suggested to have a critical role in AISs such as Behçet’s disease,Familial Mediterranean fever and Cryopyrin-associated periodic syndrome.展开更多
文摘Autoinflammatory syndrome(AIS)is characterized by periodic inflammation in the absence of infection,autoantibodies or self-reactive lymphocytes.Therefore,AIS has defining characteristics that differ from those of autoimmune diseases.The sterile inflammation observed in AIS is likely caused by the dysregulation of the innate immune system.However,the etiologies and physiopathologies of these autoinflammatory disorders remain ambiguous.Uncontrolled interleukin(IL)-1 biosynthesis,which is initiated by danger signals in the inflammasome or via nucleic acid-sensing pathways,has been suggested to have a critical role in AISs such as Behçet’s disease,Familial Mediterranean fever and Cryopyrin-associated periodic syndrome.
