Table 1.

Baseline information of the AOSD patients at enrolment

No.	G	Age	Disease duration (months)	Clinical manifestations	Previous treatments	Treatments before JAKi initiation	Treatments after enrolment	Follow-up (months)	Clinical evaluation	CR time with JAKi (months)	Present pred dose (mg/day)
1	F	33	12	Polyarthritis, rash	CTX, MTX, CsA, NSAIDs iguratimod, thalidomide,	Pred 40 mg+tocilizumab	Pred 40 mg+JAKi 5 mg two times per day	24	Effective	16	2.5
2	F	27	6	Fever, polyarthritis	/	Pred 60 mg+MTX+CsA	Pred 60 mg+MTX+ JAKi 5 mg two times per day	13	Effective	5	5
3	F	32	48	Fever, rash, sore throat, myalgia	Thalidomide	Pred 30 mg+CsA+HCQ	Pred 50 mg+HCQ+ JAKi 5 mg two times per day	12	Effective	7	5
4	F	58	24	Polyarthritis, rash	Tocilizumab	Pred 10 mg+MTX+HCQ+CsA	Pred 15 mg+MTX+HCQ +JAKi 5 mg two times per day	6	Relapse when the pred dose was reduced to 2.5 mg/day	1	/
5	F	35	24	Polyarthritis, rash	Tocilizumab, thalidomide	Pred 10 mg+MTX+HCQ+LEF	Pred 15 mg+MTX +JAKi 5 mg two times per day	1	Partially effective	/	/
6	F	29	10	Polyarthritis, early joint destruction, lymphnodemegaly, MAS	/	Pred 100 mg+MTX	Pred 60 mg+MTX +JAKi 5 mg two times per day	9	Effective	6	7.5
7	F	72	5	ESR↑	/	Pred 30 mg+HCQ	Pred 25 mg+HCQ+ JAKi 5 mg one time per day	9	Effective	3	5
8	F	25	19	Polyarthritis	CsA, HCQ	Pred 50 mg+MTX	Pred 50 mg+MTX +JAKi 5 mg two times per day	4	Partially effective	/	15
9	F	41	60	Polyarthritis	/	Pred 120 mg+MTX+NSAIDs	Pred 60 mg+MTX+ JAKi 5 mg two times per day	5	Partially effective	/	/
10	F	31	12	Polyarthritis	/	Pred 20 mg+MTX+HCQ+CsA	Pred 20 mg+MTX + HCQ+CsA +JAKi 5 mg one time per day	4	Effective	4	5
11	F	33	1	Fever, rash, sore throat, polyarthritis, myalgia	/	Pred 60 mg+MTX+HCQ	Pred 40 mg+MTX +HCQ +JAKi 5 mg two times per day	3	Effective	2	20
12	M	35	4	MAS	VP16, DX	Pred 25mg+CsA+anakinra	Pred 22.5 mg +CsA+anakinra +JAKi 5 mg two times per day	1	Partially effective	/	17.5
13	M	18	22	Polyarthritis, rash	/	Pred 20 mg+MTX	Pred 15 mg+HCQ+ JAKi 5 mg two times per day	1	Partially effective	/	10
14	F	18	10	MAS, polyarthritis, rash	NSAIDs, IVIG	Pred 50 mg+CsA+tocilizumab	Pred 50 mg+CsA+MTX +JAKi 5 mg two times per day	1	Partially effective	/	35

CR, complete remission; CsA, cyclosporine; CTX, cyclophosphamide; DX, dexamethasone; ESR, erythrocyte sedimentation rate; F, female; G, gender; HCQ, hydroxychloroquine; IVIG, intravenous immunoglobulin; JAK, Janus kinases; JAKi, JAK inhibitor, tofacitinib; LEF, leflunomide; M, male; MAS, macrophage activation syndrome; MTX, methotrexate; NSAIDs, non-steroidal anti-inflammatory drugs; Pred, prednisone; VP16, etoposide.