Table 1. Classification of the Monogenic Autoinflammatory Syndromes.

FMF: familial Mediterranean fever, MKD: mevalonate kinase deficiency, TRAPS: tumor necrosis factor receptor-associated periodic syndrome, CINCA: chronic infantile neurological cutaneous articular syndrome, FCAS: familial cold autoinflammatory syndrome, MWS: Muckle–Wells syndrome, BS: Blau syndrome, DIRA: deficiency of interleukin 1 receptor antagonist, MS: Majeed syndrome, PAPA:  pyogenic arthritis pyoderma gangrenosum and cystic acne syndrome, NLRP: NACHT domain-, leucine-rich repeat- and pyrin domain-containing protein, AD: autosomal dominant, AR: autosomal recessive [5]

	Gene	Mutated Protein	Inheritance
Monogenic periodic fevers
FMF	MEFV	Pyrin/marenostrin	AR
MKD	MVK	Mevalonate kinase	AR
TRAPS	TNFRSF1A	TNFRSF1A	AD
Cryoporin-associated periodic syndromes
CINCA	NRLP3/CIAS1	Cryopyrin	AD, sporadic
FCAS			AD
MWS			AD
NLRP12-associated autoinflammatory disorder	NLRP12	NLRP12	AD
Autoinflammatory granulomatous disorders
BS	NOD2/CARD15	NOD 2(CARD15)	AD
Early-onset sarcoidosis	NOD2/CARD15	NOD 2(CARD15)	Sporadic
Autoinflammatory pyogenic disorders
DIRA	IL1RN	Interleukin 1 receptor antagonist	AR
MS	LPIN2	Lipin-2	AR, sporadic
PAPA	PSTPIP1 (CD2BP1)	PSTPIP1 (CD2BP1)	AD