Pediatric Autoinflammatory Bone Disorders-a Mini Review with Special Focus on Pathogenesis and Inborn Errors of Immunity

Overview

Journal Front Pediatr

Specialty Pediatrics

Date 2023 Jun 21

PMID 37342528

Authors

Rebecca Hetrick

Melissa Oliver

Affiliations

Soon will be listed here.

Abstract

Autoinflammatory bone disorders are a group of diseases characterized by sterile osteomyelitis. This includes chronic nonbacterial osteomyelitis and the monogenic forms, Majeed syndrome and deficiency of the interleukin-1 receptor antagonist. These disorders result from innate immune system dysregulation and cytokine imbalance that triggers inflammasome activation causing downstream osteoclastogenesis and excessive bone remodeling. In this review, we will summarize the immunopathogenesis of pediatric autoinflammatory bone diseases with a special focus on the genetics and inborn errors of immunity, while briefly touching on the clinical manifestations and management of each disease as well as areas for future research.

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