Genetics of somatic auto-inflammatory disorders

Systemic autoinflammatory disorders (SAIDs) encompass a heterogeneous group of monogenic disorders characterised by recurrent episodes of systemic and organ-specific inflammation. Genetic studies have facilitated the identification of Mendelian forms of SAIDs but many patients still remain without a diagnosis. Recent studies have uncovered that somatic (acquired) mutations can cause later-onset SAIDs. In this review, we will discuss the current knowledge surrounding the genetics of these acquired auto-inflammatory disorders (AAIDs), with a focus on VEXAS, NLRP3-associated AAIDs and Schnitzler's syndrome and provide suggestions for future research in this field.