Aetiology
Aetiology is not known. Family studies show a possible genetic predisposition.[11] Increased inflammation and immunological mechanisms play a role, but it is not clear whether it is an auto-immune or auto-inflammatory disorder.[7] Auto-inflammatory disorders are a group of heritable diseases characterised by recurrent episodes of systemic inflammation in the absence of an obvious auto-immune pathology such as pathogenic high-titre auto-antibodies or antigen-specific T cells. Unlike other auto-immune diseases, Behcet's syndrome is not typically associated with auto-antibodies, Raynaud's disease, Sjogren's syndrome, thrombocytopenia, haemolytic anaemia, sun hypersensitivity, serosal involvement, or increased risk of other auto-immune diseases. Some features that separate it from auto-inflammatory conditions include tendency to abate with time, absence of mutations associated with auto-inflammatory diseases, and higher prevalence than typical auto-inflammatory diseases such as familial Mediterranean fever. Both innate and adaptive immune systems may be involved.[12]
Pathophysiology
Pathogenesis is unknown. There is neutrophil hyper-reactivity, 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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