TABLE 3.

Features of KS and Recurrent Fever Syndrome^5,20,21

Syndrome	Duration	Positive Diagnostic Criteria	Standard/Reported Treatments	Genetics
Classical Kawasaki syndrome	Single episode or recurrent, >5 d	Four of the following symptoms: bilateral conjunctival injection, oral mucosal changes, polymorphous rash, erythema/edema of palms/soles or desquamation of fingertips/toes, cervical adenopathy	IVIg infusion, TNF inhibitorsa	No known genetic mutation
PFAPA syndrome	Recurrent, 3–5 d, regular intervals	Aphthous stomatitis, pharyngitis, cervical lymphadenitis; exclusion of bacterial infection/cyclic neutropenia	Corticosteroids, colchicine, tonsillectomya	No known genetic mutation
Hyperimmunoglobulin D syndrome (HIDS) with periodic fever	Recurrent, 3–7 d	Fever, abdominal pain, oral ulcers, arthralgia, vomiting, diarrhea	Anakinraa	Autosomal recessive defect in MVK affecting mevalonic kinase
TNF receptor-associated periodic syndrome (TRAPS)	Recurrent, >1 wk	Fever, abdominal pain, centrifugal rash, periorbital edema, migratory arthralgia	Corticosteroids, anakinra, etanercept	Autosomal dominant defect in TNFRS1A gene affecting TNFR
Familial Mediterranean fever (FMF)	Recurrent, 2 d	Sterile peritonitis with abdominal pain, monoarthritis, myalgia, erysipelas-like erythema (shins/foot)	Colchicine, Anakinraa	Autosomal recessive defect in MEFV, affecting pyrin
Familial cold autoinflammatory syndrome (FCAS)	Recurrent, <1 d	Arthralgia, cold-induced urticarial-like rash, conjunctivitis	Anakinra, rilonacept, canakinumab	Autosomal dominant defect in NLRP3, affecting cryopyrin
Muckle-Wells syndrome (MWS)	Recurrent, 1–2 d	Arthralgia, generalized urticarial-like rash, conjunctivitis, sensorineural hearing loss	Anakinra, rilonacept, canakinumab	Autosomal dominant defect in NLRP3, affecting cryopyrin
Neonatal-onset multisystem inflammatory disease (NOMID)	Chronic	Neonatal onset, hepatosplenomegaly, arthropathy, generalized urticarial-like rash, uveitis, chronic aseptic meningitis, sensorineural hearing loss	Anakinra	Autosomal dominant, often sporadic defect in NLRP3, affecting cryopyrin

TNF indicates tumor necrosis factor.

^aReported therapy needing futher investigation.