Patient no. | Sex | Disease duration (years) | Age (years) | Main symptoms at anakinra treatment initiation | Previous treatments (DMARDs) | Prednisone (mg/kg/day) | Associated DMARDs | Response (month 3; last follow-up) | Follow-up on anakinra (months) | Comments |
SoJIA | ||||||||||
1 | M | 5.3 | 10.5 | Fever, arthritis, pericarditis | MTX, Thal | 0.4 | CR; CR | 27 | Stopped steroids (M20) | |
2 | F | 12.3 | 22.5 | Fever, rash, arthritis pericarditis, pleuritis | MTX, ETA, INF, Thal | 1 | CR; CR | 25 | Disease flare (M21), CR on anakinra 100 mg twice a day | |
3 | M | 12.3 | 16.2 | Arthritis, rash, myalgia | MTX, CS, ETA, CP | 0.3 | CR; CR | 22 | ||
4 | M | 5.4 | 9.1 | Fever, rash, arthralgia | MTX, ETA | 0.4 | CR; PR | 17 | ||
5 | F | 0.8 | 11.1 | Fever, arthritis, rash | MTX, ETA | 0.1 | CR; CR | 12 | ||
6 | F | 5.3 | 9.4 | Rash, arthritis | MTX, CS, ETA | 0.6 | CR;– | 6 | Leishmaniasis (M6) | |
7 | M | 6.1 | 17.8 | Arthritis | MTX, ETA | 0.1 | PR; PR | 25 | Ancient, diffuse ankyloses | |
8 | M | 15.8 | 22.9 | Fever, arthritis, rash | MTX, Thal | 0.3 | MTX (0.6 mg/kg/week) | PR; PR | 19 | |
9 | F | 8.7 | 15.9 | Arthritis prosthesis | MTX, ETA, CS | 0.6 | PR; PR | 18 | Painful hip awaiting prosthesis | |
10 | F | 9.4 | 18.2 | Arthritis | MTX, CS, ETA | 0.2 | PR; PR | 13 | ||
Non-responders/v. transient response | ||||||||||
11 | F | 3.7 | 7.1 | Rash, arthritis | MTX, ETA, INF, CS, Ritu | 0.4 | NR; NR | 24 | ||
12 | F | 6.4 | 9.3 | Fever, arthritis | MTX, ETA | 0.8 | PR; NR | 21 | ||
13 | F | 4.5 | 12.4 | Arthritis | MTX, Thal, | 0.4 | AZA, INF, CS | NR; NR | 22 | |
14 | F | 1.7 | 5.2 | Fever, arthritis, rash | MTX, Thal, ETA, INF | 0.5 | MTX (0.6 mg/kg/week) | NR; NR | 13 | Varicella (M13) |
15 | M | 6.8 | 8.7 | Arthritis | MTX | 1.3 | NR; NR | 7 | ||
16 | M | 0.8 | 2.9 | Fever, arthritis, myalgia | 1.2 | NR; NR | 7 | |||
17 | F | 3.3 | 10.0 | Arthritis | MTX | 0.2 | MTX (0.6 mg/kg/week) | NR; NR | 6 | |
18 | F | 5.7 | 9.4 | Fever, arthritis pericarditis | MTX | 0.4 | NR; NR | 6 | ||
19 | M | 11.6 | 14.5 | Arthritis | MTX, ETA | 0.2 | NR at 2 months | 2 | ||
20 | F | 7.9 | 13.5 | Arthritis | MTX, ETA | 0.1 | MTX (0.6 mg/kg/week) | NR at 2 months | 2 | |
AoSD | ||||||||||
21 | F | 8 | 31 | Fever, arthritis, rash, myalgia, pharyngitis | MTX, ETA, INF | 0 | MTX (7.5 mg/week) | CR; CR | 23 | Anakinra interrupted for bronchitis (M8) and for pregnancy wish (M13) |
22 | F | 2 | 22 | Fever, arthritis, rash pharyngytis | MTX, INF | 25 | MTX (10 mg/week) | CR; CR | 22 | Varicella, MAS (M16) transient anakinra stopped |
23 | F | 10 | 26 | Arthritis | MTX, ETA, SLZ, LEF | 20 | MTX (12.5 mg/week) | CR; CR | 21 | Hepatitis A (M3) |
24 | M | 7 | 35 | Fever, arthritis | MTX, INF, ADA, Ritu, ETA, Thal | 20 | MTX (20 mg/week) | CR; CR | 19 | Hip osteonecrosis |
25 | M | 4 | 32 | Fever, arthritis, myalgia | MTX, ETA, Ritu, IVIG, ADA | 30 | MMF, Col | CR; CR | 16 | MMF stopped |
26 | M | 5 | 51 | Fever, arthritis, myalgia, pharyngitis | MTX, ETA, INF, AZA, MMF, IVIG, CP | 10 | CR; CR | 16 | Predenisone stopped (M8) | |
27 | M | 7 | 31 | Arthritis | MTX | 0 | MTX (15 mg/week) | CR; CR | 12 | Local infection following piercing (M10) |
28 | F | 9 | 34 | Fever, arthritis, myalgia pharyngitis, pericarditis | MTX, INF, ETA | 40 | MTX (15 mg/week) | CR; CR | 11 | |
29 | F | 27 | 50 | Fever, arthritis, rash myalgia, adenopathy | MTX | 80 | MTX (17.5 mg/week) | CR; CR | 11 | Stopped prednisone (M3) |
30 | F | 4 | 62 | Fever, arthritis, rash, myalgia | MTX, SLZ, INF | 40 | PR; PR | 27 | ||
31 | F | 3 | 47 | Fever, arthritis, rash | MTX | 30 | Col | PR; PR | 15 | |
Non-responders | ||||||||||
32 | F | 5 | 62 | Fever, arthritis, pleuresis | MTX, INF, IVIG | 40 | MTX (20 mg/week) | NR; NR | 9 | Anakinra stopped for lack of efficacy (M8) |
33 | F | 4 | 24 | Fever, arthritis, rash, myalgia, pharyngytis, adenopathy | MTX, IVIG | 20 | MTX (20 mg/week) | NR; NR | 8 | Only transient efficacy |
34 | F | 18 | 40 | Fever, arthritis, myalgia, rash | MTX, Thal, ETA | 0 | Intolerance | 1 | Severe skin rash (day 10) | |
35 | F | 4 | 25 | Fever, arthritis, rash pharyngitis | MTX, IVIG | 20 | MTX (20 mg/week) | Intolerance | <1 | Anakinra 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.