Autoinflammatory syndrome (AIDs) – is a very rare inflammatory diseases group associated with abnormal regulation of innate immunity. AIDs clinical symptoms diversity complicates their diagnosis. As these syndromes occur at an early age and causes severe impairment of life quality, it is important to recognite early , diagnose, treat them and avoid complicated, difficult cases. Periodic, recurrent episodes of fever and positive family history are the first symptoms suggestive of AIDs. By submitting this clinical case, complexity of diagnosis and a variety of symptoms are emphasized.. AIDs treatment is difficult, because there is no clear treatment guidelines and no enough research to evaluate the effectiveness of treatment. The main drugs are biologics??ar taip sakoma?. After the analysis of the recent studies, interleukin (IL) 1β receptor antagonist – Anakinra and Canakinumab – IL-1β monoclonal antibody are the most effective.Currently, these drugs provide the greatest hope in the treatment of AIDs.
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