Table 1

Baseline information of the AOSD patients at enrolment

No.GAgeDisease duration (months)Clinical manifestationsPrevious treatmentsTreatments before
JAKi initiation
Treatments after enrolmentFollow-up
Clinical evaluationCR time with JAKi (months)Present pred dose (mg/day)
1F3312Polyarthritis, rashCTX, MTX, CsA, NSAIDs iguratimod, thalidomide,Pred 40 mg+tocilizumabPred 40 mg+JAKi 5 mg two times per day24Effective162.5
2F276Fever, polyarthritis/Pred 60 mg+MTX+CsAPred 60 mg+MTX+ JAKi 5 mg two times per day13Effective55
3F3248Fever, rash, sore throat, myalgiaThalidomidePred 30 mg+CsA+HCQPred 50 mg+HCQ+ JAKi 5 mg two times per day12Effective75
4F5824Polyarthritis, rashTocilizumabPred 10 mg+MTX+HCQ+CsAPred 15 mg+MTX+HCQ
+JAKi 5 mg two times per day
6Relapse when the pred dose was reduced to 2.5 mg/day1/
5F3524Polyarthritis, rashTocilizumab, thalidomidePred 10 mg+MTX+HCQ+LEFPred 15 mg+MTX
+JAKi 5 mg two times per day
1Partially effective//
6F2910Polyarthritis, early joint destruction, lymphnodemegaly, MAS/Pred 100 mg+MTXPred 60 mg+MTX
+JAKi 5 mg two times per day
7F725ESR↑/Pred 30 mg+HCQPred 25 mg+HCQ+ JAKi 5 mg one time per day9Effective35
8F2519PolyarthritisCsA, HCQPred 50 mg+MTXPred 50 mg+MTX
+JAKi 5 mg two times per day
4Partially effective/15
9F4160Polyarthritis/Pred 120 mg+MTX+NSAIDsPred 60 mg+MTX+ JAKi 5 mg two times per day5Partially effective//
10F3112Polyarthritis/Pred 20 mg+MTX+HCQ+CsAPred 20 mg+MTX + HCQ+CsA
+JAKi 5 mg one time per day
11F331Fever, rash, sore throat, polyarthritis, myalgia/Pred 60 mg+MTX+HCQPred 40 mg+MTX +HCQ
+JAKi 5 mg two times per day
12M354MASVP16, DXPred 25mg+CsA+anakinraPred 22.5 mg +CsA+anakinra
+JAKi 5 mg two times per day
1Partially effective/17.5
13M1822Polyarthritis, rash/Pred 20 mg+MTXPred 15 mg+HCQ+ JAKi 5 mg two times per day1Partially effective/10
14F1810MAS, polyarthritis, rashNSAIDs, IVIGPred 50 mg+CsA+tocilizumabPred 50 mg+CsA+MTX
+JAKi 5 mg two times per day
1Partially 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.