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Abstract The autoinflammatory disorders are a new and expanding classification of inflammatory diseases characterized by recurrent episodes of systemic inflammation in the absence of pathogens, autoantibodies or antigen specific T cells. These diseases are generally compatible with normal life expectancy, bar the significant risk of developing AA amyloidosis. Recent insights into their molecular pathogenesis with identification of susceptibility genes and characterisation of new proteins and pathways have led to improved diagnosis and development of therapies. NSAIDs, colchicine and corticosteroids have role in the treatment of some of these disorders, but biologic drugs that target interleukin-1β as anakinra are emerging as consistently effective therapies |