“FMF-like” (Meet Tel-Hashomer criteria but atypical additional features)
1515 / 4 y / Y / Abdominal pain, pleuritis, cervical lymphadenopathy, pharyngitis / 3 days / Y-beneficial / No
"PFAPA-LIKE" SYMPTOMS
1508 / 5 y / Y / Aphthous ulcers, pharyngitis, cervical lymphadenopathy. pleuritis / Up to 7 days / No / No
1495 / 2 y / Y / Apthhous ulcers, pharyngitis, cervical lymphadenopathy. / 3-5 days / No / No
1650 / Infancy / Y / Aphthous ulcers, pharyngitis, cervical lymphadenopathy. / Up to 7 days / Y- minimal benefit / No
1534 / 10 mo / Y / Pharyngitis, cervical lymphadenopathy. / 3-7 days / No / No
1087 / 2 y / Y / Aphthous ulcers, pharyngitis, maculopapular rash. / 5 days / No / No
1190 / 2 mo / Y / Aphthous ulcers, pharyngitis, cervical lymphadenopathy. / 3 days / Y* / No
1338 / 6 mo / Y / Pharyngitis, cervical lymphadenopathy / 4-5 days / No / No
PREDOMINANT SEROSITIS SYMPTOMS
1518 / Infancy / Y / Pleuritis, cervical lymphadenopathy, pharyngitis, aphthous ulcers. / 4 days / Y-minimal benefit / Etanercept
861 / Infancy / Y / Pleurisy, cervical lymphadenopathy / 1 week / No / No
952 / 17y / Y / Pleuritis, pericarditis, / 3-10 days / No / Etanercept
UVEITIS
1490 / 6 y / Y / Uveitis, nephropathy, diarrhea / 3-21 days / No / Etanercept
1119 / 10 y / Y / Uveitis, Crohn’s disease, erythema nodosum / 3-5 days / No / Infliximab
JOINT SYMPTOMS
1496 / 1 y / Y / Systemic-onset Juvenile Inflammatory Arthritis, aphthous ulcers. / 2-3 months / No / Etanercept
1222 / 5 y / Y / Juvenile Inflammatory Arthritis / 12 hours / No / No
OTHER
1568 / 11y / Y / Arthralgia / 3-5 days / No / No
809 / 55y / Y / Arthralgia, myalgia, cervical lymphadenopathy / 2-4 weeks / Y-Ineffective / Anakinra
1554 / 14y / Y / Erysipeloid erythema, cervical lymphadenopathy / 3 days / Y-beneficial / No
1668 / 3y / Y / Abdominal pain, myalgia / 4 days / No / No
1511 / 20y / N / Arthralgia, aphthous ulcers / 3 days / Y-beneficial / No
968 / 24y / Y / Recurrent aseptic meningitis / 2-3 days / No / No
858 / 16y / Y / Erysipeloid erythema, arthralgia, cervical lymphadenopathy, diarrhea / 3 days / No / No
800 / 14y / Y / Abdominal pain / 3-5 days / Y-beneficial / No
964 / 4 mo / Y / Myalgia, cervical lymphadenopathy / 3 days / No / No
876 / 15 mo / Y / Erysipeloid erythema, aphthous ulcers, cervical lymphadenopathy / 3-4 days / No / No
1216 / 52y / Y / Erysipeloid erythema, arthralgia / 1-2 days / No / No
780 / 6 mo / Y / Arthralgia / <1 day / Y-beneficial / No
1084 / 22y / Y / Arthralgia, cervical lymphadenopathy / 3-5 days / Y-beneficial / No
905 / 7 y / Y / Fever alone / 1 day / No / No
896 / 7 y / Y / Pharyngitis / 14-20 days / No / No
979 / 16 mo / Y / Diarrhea / 2 days / No / No
1260 / 3 y / Y / Abdominal pain, arthralgia / 1-2 days / No / No
883 / 4 y / Y / Myalgia, aphthous ulcers, conjunctivitis / 3-4 days / No / No
Supplementary Table- Clinical features of mutation negative patients.