|
|
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
|
