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

From: Recurrent fever: diagnostic procedure

  FMF TRAPS MKD/ HyperIgD PFAPA
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