Abstract
A disease, which repeats inflammations but is not based on infections or autoimmunity, was recently defined as autoinflammatory diseases. The autoinflammatory diseases 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. This disease is developed by the abnormality of molecules which regulate inflammation, innate immunity and apoptosis, and patients have been suffered with treatment by the difficulty of making a diagnosis. However, a diagnosis is comparatively easy by their presenting the characteristic clinical observation. In addition, genetic analysis can put the diagnosis on a firm basis. Giving that therapies for individual disorder of autoinflammatory diseases are different, we always put these disorders in mind when we saw patients who have unknown persistent inflammation.