Table 1 Main clinical manifestations during fever episodes in patients with periodic fever syndrome and genes responsible of hereditary periodic syndrome.

Abdominal pain

+++

+++

++

-

Diarrhea

+/-

-

+++

-

Arthritis

+++

-

+++

Lymphadenopathy

+/-

++

+++

+++

Rash

+/-

++

+++

-

Stomatitis

-

-

-

+++

Pharyngitis

-

-

-

+++

Splenomegaly

++

-

+++

+/-

Conjunctivitis

-

+++

-

-

Duration of fever

1-4 Days

Variable >1 week

2-5 Days

2-8 Days

Gene/chromosome

MEFV16p13.3

TNFRSF1A12p13

MVK 12q24

unknown

Protein

Pyrin

TNFR1

MVK

unknown

Inheritance

AR

AD

AR

none