ABSTRACT

Recurrent fever is common in young, especially preschool, children, and is associated with repeated episodes of fever that, in some cases, may last for weeks. Most often, these episodes are due to repeated infections; however, when there is a periodic recurrence, they can also be because of conditions such as autoinflammatory diseases (AIDs). Since their discovery nearly two decades ago, AIDs received a growing interest in research studies that not only increased our understanding of the AID pathologies, but also enriched the literature on innate immune responses, including inflammasome-mediated pathways. Inflammasomes are protein complexes formed in response to the activation of a group of intracellular pathogen-recognition receptors by a variety of inducers originating from the infection or cellular stress. They are involved in the generation of caspase-1, which is required for the generation of active interleukin (IL)-1β and IL-18, and were also shown to induce the generation of a wide variety of other cytokines, including tumor necrosis factor (TNF)-α, IL-6, and interferons (IFNs). This review aims not only to raise awareness about pediatric AIDs with periodic fever, but also to present the current literature about the molecular basis of inflammasome-mediated pathways involved in the disease pathogenesis. Future studies that would further enlighten the relationship between inflammasome-mediated pathways and AIDs would contribute to the development of more effective treatment strategies aiming to improve the patient life quality and help to avoid long-term complications.