Abstract
Periodic fever syndrome is characterized by three or more febrile episodes in six months, each occurring at least seven days apart. Immune dysregulation in systemic autoinflammatory syndromes involves innate immunity (neutrophils, monocytes, and macrophages) and cytokines, mainly IL-1, with tumor necrosis factor (TNF), interferon alpha and beta, IL-2, IL-12, IL-18, and IL-23. Treatment includes colchicine, corticosteroids, immunosuppressants, and biologics. Prebiologic screening should be done to rule out tuberculosis, HIV, hepatitis B, and hepatitis C. Monitoring includes biomarkers of inflammation, disease activity score, looking for disease-specific organ involvement/complications, and treatment-related adverse drug reactions. There are research gaps in determining standardized treatment for the majority of autoinflammatory syndromes, duration of treatment, novel targets for treatment, and long-term prognosis.