In addition to above-described inflammasomopathies in FMF and CAPS, other underlying mechanisms for SAIDs can be summarised as follows: intracellular stress that causes production of reactive oxygen species; aberrant apoptosis; protein-misfolding and aberrant cytokine production as in TRAPS and HIDS; increased interferon signalling as in SAVI and CANDLE; NF-κB activation disorder as in Blau syndrome; and deficiency of enzymes such as adenosine deaminase 2 causing autoinflammation and vasculitis (2, 3) (Table 1). Here, NFKB1 is linked to familial Mediterranean fever.