Periodic fever syndrome

Background

• Most of the periodic fever syndromes result in caspase-1 activation and the release of IL-1β

	Ethnicity	Age at Onset	Duration	LNs	AP	MSK	CP	Rash	Other	Treatment
FMF	Armenian, Arab, Jewish, Turkish	<20 years	1-4 days	rare	very common sterile peritonitis	monoarthritis of knee, ankle, wrist	pleuritis, often unilateral	rare erysipelas-like on legs	pericarditis, scrotal attacks, splenomegaly	colchicine
HIDS	Dutch, French	childhood	3-7 days	cervical	very common	arthralgias, rare arthritis	unusual	very common, maculopapular	hepatosplenomegaly, aphthous ulcers	etanercept, IL-1 inhibitors
NAPS12	Guadeloupe, Italy	neonatal	2-10 days	yes	yes	arthralgias, myalgias	yes	urticaria/erythema	cold sensitivity, headache	—
TRAPS	mostly northern European, but all ethnic groups	variable	more than 5 days, up to weeks to months	common	common	arthralgias, myalgias	yes	common erysipelas-like including arms	periorbital edema, headache	IL-1 inhibitors, TNF-α inhibitors, anti-IL6R mAb
CAPS	all ethnic groups	childhood/neonatal	variable, depending on subtype	very rare	rarerare	arthralgias, arthritis, bony overgrowth (depending on subtype)	no	urticaria, erythema	cold sensitivity, deafness, aseptic meningitis, papilledema, dysmorphism (depending on subtype)	IL-1 inhibitors
PFAPA	all ethnic groups	<5 years	3 to 6 days	cervical	common	arthralgias	rare	common	aphthous stomatitis, pharyngitis, headache	corticosteroids, cimetidine, tonsillectomy

• LNs=lymph nodes; AP=abdominal pain; MSK=musculoskeletal symptoms; CP=chest pain
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