Decoding Autoinflammatory Diseases: A Concise Overview

Systemic autoinflammatory diseases are rare genetic conditions caused by dysregulation of the innate immune system, leading to recurrent or persistent inflammation. Advances in genomics have significantly reinforced our understanding of these diseases, revealing complex genetic causes involving both germline and somatic variations. More than 55 distinct forms of monogenic autoinflammatory disorders (AIDs) have been described till date. This short review is an attempt to highlight the clinical features, genetic basis, and major inflammatory signaling pathways, which aims to provide a fundamental understanding of monogenic AIDs.