Table 1.

Current European Medicines Agency and US Food-and-Drug Administration drug approval status for autoinflammatory diseases

Treatment	L	European Medicines Agency (EMA)	Food-and-Drug Administration (FDA)
CAPS
Canakinumab	1B	CINCA/NOMID, MWS and severe FCAS ≥2 years and ≥ 7.5 kg	FCAS and MWS ≥ 4 years
Rilonacept	1B	none	FCAS and MWS ≥ 12 years
Anakinra	2A	all CAPS patients ≥8 months and ≥ 10 kg	NOMID/CINCA only
TRAPS
Canakinumab	1B	TRAPS ≥2 years and ≥ 7.5 kg	TRAPS in all adults and children
Anakinra	2B	none	none
Etanercept	2B	none	none
HIDS (MKD)
Canakinumab	1B	MKD ≥2 years and ≥ 7.5 kg	MKD in all adults and children
Anakinra	2B	none	none
Etanercept	3B	none	none
Adalimumab	3B	none	none
Tocilizumab	4	none	none

The table summarizes indications and specifications for autoinflammatory drug approval as of July 2019. Evidence levels were adapted from the Oxford Centre for Evidence-based Medicine levels of evidence and grades of recommendation [24]: 1B individual randomised controlled trial; 2A systematic review of cohort studies; 2B individual cohort study; 3B, individual case-control study, non-consecutive cohort study; 4 case series