Etiopathogenetic hypotheses regarding the autoinflammatory syndromes
• Many of these syndromes are identified to be monogenetic disorders of the inflammatory cascade. Genetic culprits for several of these conditions have been identified in recent years and likely will continue to emerge.
• Altered innate immune functions leading to cytokine overproduction or increased activity is thought to play a critical role.
• Dysregulation of the assembly and function of the inflammasome, ultimately leading to the excess production of IL-1β, is an important mechanism in several autoinflammatory syndromes. Disruption can occur at many different places, each corresponding to specific syndromes.