Auto-inflammatory diseases (AIDs) are defined as dysfunction of innate immunity [1]. They can involve different pathways. Familial Mediterranean fever (FMF), Still's disease, Mevalonate Kinase Deficiency (MKD), Cryopyrin-associated periodic syndrome (CAPS) and PSTPIP1-associated AIDs are associated with inflammasome activation, whereas, A20 haploinsufficiency (HA20) involves more likely NF-kB pathway activation. On the contrary, interferonopathies, such as Aicardi-Goutières syndrome (AGS) and SAVI (STING-associated vasculopathy with onset in infancy), are driven by dysregulated type I interferon signaling, while actinopathies, including Wiskott-Aldrich syndrome (WAS) and ACTB mutations, result from aberrations in the actin cytoskeleton, leading to immune dysregulation and autoinflammatory features [1].
