# Updates in the Management of Hereditary Periodic Fever Syndromes in Children

## TL;DR

This paper discusses recent developments in treating hereditary periodic fever syndromes in children, focusing on immune dysregulation and treatment options.

## Contribution

The paper highlights current treatment strategies and identifies research gaps in managing autoinflammatory syndromes.

## Key findings

- Immune dysregulation in these syndromes involves innate immunity and specific cytokines like IL-1 and TNF.
- Treatment options include colchicine, corticosteroids, immunosuppressants, and biologics.
- Research gaps remain in determining standardized treatment and long-term prognosis.

## 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.