Etiology
Etiology is not known. Family studies show a possible genetic predisposition.[11] Increased inflammation and immunologic mechanisms play a role, but it is not clear whether it is an autoimmune or autoinflammatory disorder.[7] Autoinflammatory disorders are a group of heritable diseases characterized by recurrent episodes of systemic inflammation in the absence of an obvious autoimmune pathology such as pathogenic high-titer autoantibodies or antigen-specific T cells. Unlike other autoimmune diseases, Behcet syndrome is not typically associated with autoantibodies, Raynaud disease, Sjogren syndrome, thrombocytopenia, hemolytic anemia, sun hypersensitivity, serosal involvement, or increased risk of other autoimmune diseases. Some features that separate it from autoinflammatory conditions include tendency to abate with time, absence of mutations associated with autoinflammatory diseases, and higher prevalence than typical autoinflammatory diseases such as familial Mediterranean fever. Both innate and adaptive immune systems may be involved.[12]
Pathophysiology
Pathogenesis is unknown. There is neutrophil hyperreactivity, but it is not clear whether this is primary or secondary to cytokine-directed activation.[12] Evidence from retrospective patient cohort analyses suggests that there may be different clusters of disease presentation, and each may have a different pathogenesis.[13]
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