Autoinflammatory uveitis

AbstractAutoinflammatory syndromes are a new group of distinct hereditable disorders characterized by episodes of seemingly unprovoked inflammation (most commonly in skin, joints, gut, and eye), the absence of a high titer of auto‐antibodies or auto‐reactive T cells, and an inborn error of innate immunity.Ocular involvement is frequent in the monogenic autoinflammatory disorders and generally occurs as spontaneously recurring inflammatory events at different ocular sites caused by aberrant release of proinflammatory cytokines, mainly IL‐1β.Innate and adaptive immune systems have evolved together to protect the host by quickly responding to danger signals, eliminating pathogens and creating immunological memory as well as immunological tolerance to self. Loss of control mechanisms and/or regulation of either the adaptive or the innate immune system lead to autoimmunity and autoinflammation respectively.We present the clinical case of a family with three members affected with the same form of uveitis, that we have considered as presumed autoinflammatory uveitis.As from this case we discuss about pathogenesis and therapy of autoinflammatory diseases and uveitis.