Table 2 Patients’ characteristics and treatment at the onset and at the latest follow-up of anakinra treatment
Patient no.SexDisease duration (years)Age (years)Main symptoms at anakinra treatment initiationPrevious treatments (DMARDs)Prednisone (mg/kg/day)Associated DMARDsResponse (month 3; last follow-up)Follow-up on anakinra (months)Comments
SoJIA
1M5.310.5Fever, arthritis, pericarditisMTX, Thal0.4CR; CR27Stopped steroids (M20)
2F12.322.5Fever, rash, arthritis pericarditis, pleuritisMTX, ETA, INF, Thal1CR; CR25Disease flare (M21), CR on anakinra 100 mg twice a day
3M12.316.2Arthritis, rash, myalgiaMTX, CS, ETA, CP0.3CR; CR22
4M5.49.1Fever, rash, arthralgiaMTX, ETA0.4CR; PR17
5F0.811.1Fever, arthritis, rashMTX, ETA0.1CR; CR12
6F5.39.4Rash, arthritisMTX, CS, ETA0.6CR;–6Leishmaniasis (M6)
7M6.117.8ArthritisMTX, ETA0.1PR; PR25Ancient, diffuse ankyloses
8M15.822.9Fever, arthritis, rashMTX, Thal0.3MTX (0.6 mg/kg/week)PR; PR19
9F8.715.9Arthritis prosthesisMTX, ETA, CS0.6PR; PR18Painful hip awaiting prosthesis
10F9.418.2ArthritisMTX, CS, ETA0.2PR; PR13
Non-responders/v. transient response
11F3.77.1Rash, arthritisMTX, ETA, INF, CS, Ritu0.4NR; NR24
12F6.49.3Fever, arthritisMTX, ETA0.8PR; NR21
13F4.512.4ArthritisMTX, Thal,0.4AZA, INF, CSNR; NR22
14F1.75.2Fever, arthritis, rashMTX, Thal, ETA, INF0.5MTX (0.6 mg/kg/week)NR; NR13Varicella (M13)
15M6.88.7ArthritisMTX1.3NR; NR7
16M0.82.9Fever, arthritis, myalgia1.2NR; NR7
17F3.310.0ArthritisMTX0.2MTX (0.6 mg/kg/week)NR; NR6
18F5.79.4Fever, arthritis pericarditisMTX0.4NR; NR6
19M11.614.5ArthritisMTX, ETA0.2NR at 2 months2
20F7.913.5ArthritisMTX, ETA0.1MTX (0.6 mg/kg/week)NR at 2 months2
AoSD
21F831Fever, arthritis, rash, myalgia, pharyngitisMTX, ETA, INF0MTX (7.5 mg/week)CR; CR23Anakinra interrupted for bronchitis (M8) and for pregnancy wish (M13)
22F222Fever, arthritis, rash pharyngytisMTX, INF25MTX (10 mg/week)CR; CR22Varicella, MAS (M16) transient anakinra stopped
23F1026ArthritisMTX, ETA, SLZ, LEF20MTX (12.5 mg/week)CR; CR21Hepatitis A (M3)
24M735Fever, arthritisMTX, INF, ADA, Ritu, ETA, Thal20MTX (20 mg/week)CR; CR19Hip osteonecrosis
25M432Fever, arthritis, myalgiaMTX, ETA, Ritu, IVIG, ADA30MMF, ColCR; CR16MMF stopped
26M551Fever, arthritis, myalgia, pharyngitisMTX, ETA, INF, AZA, MMF, IVIG, CP10CR; CR16Predenisone stopped (M8)
27M731ArthritisMTX0MTX (15 mg/week)CR; CR12Local infection following piercing (M10)
28F934Fever, arthritis, myalgia pharyngitis, pericarditisMTX, INF, ETA40MTX (15 mg/week)CR; CR11
29F2750Fever, arthritis, rash myalgia, adenopathyMTX80MTX (17.5 mg/week)CR; CR11Stopped prednisone (M3)
30F462Fever, arthritis, rash, myalgiaMTX, SLZ, INF40PR; PR27
31F347Fever, arthritis, rashMTX30ColPR; PR15
Non-responders
32F562Fever, arthritis, pleuresisMTX, INF, IVIG40MTX (20 mg/week)NR; NR9Anakinra stopped for lack of efficacy (M8)
33F424Fever, arthritis, rash, myalgia, pharyngytis, adenopathyMTX, IVIG20MTX (20 mg/week)NR; NR8Only transient efficacy
34F1840Fever, arthritis, myalgia, rashMTX, Thal, ETA0Intolerance1Severe skin rash (day 10)
35F425Fever, arthritis, rash pharyngitisMTX, IVIG20MTX (20 mg/week)Intolerance<1Anakinra stopped (M1) (injection site reactions)
  • ADA, adalimumab; AoSD, adult-onset Still disease; AZA, azathioprine; Col, colchicine; CP, cyclophosphamide; CR, complete response: no systemic symptoms and at least 50% improvement of ACR score (AoSD) or ACR pediatric score (SoJIA); CS, cyclosporin; DMARDs, disease-modifying antirheumatic drugs; ETA, etanercept; INF, infliximab; IVIG, intravenous immunoglobulins; M(n), month no.; MAS, macrophage activation syndrome; MMF, mycophenolate mofetil; MTX, methotrexate; NR, non-responder; PR, partial response: no systemic symptoms and 20 to 49% improvement of ACR score (AoSD) or 30 to 49% improvement of ACR pediatric score (SoJIA); Ritu, rituximab; SLZ, sulphasalazin; SoJIA, Systemic-onset juvenile idiopathic arthritis; Thal, thalidomide.