Table I.

Clinical features and treatment of reported patients

Feature	Case 1	Case 2	Case 3	Case 4
Gender	Male	Female	Female	Female
Age, years	3	6	12	1 year 4 months
Quotidian fever	Yes	Yes	Yes	Yes
Typical evanescent rash	Yes	Yes	No	Yes
Hepatomegaly	Yes	Yes	Yes	Yes
Splenomegaly	Yes	No	Yes*	Yes*
Lymphadenopathy	Yes	Yes	Yes*	Yes
Pericarditis	Yes*	Yes	Yes*	No
Pleuritis	No	Yes	No	No
Abdominal pain	Yes	No	No	No
Arthritis	Yes	Yes	Yes*	No
Arthralgia	Yes	Yes	Yes	Yes
MAS	Yes	No	No	No
Treatment to achieve remission	MPS IV, MPS PO	MPS IV pulse, MPS PO, MTX, tocilizumab	MPS PO, MTX	MPS IV, MPS PO
Flares	Yes	Yes	No	Yes

MPS IV – methylprednisolone intravenous, MPS PO – methylprednisolone per os, MTX – methotrexate.

^*Signs were revealed by instrumental examination.