Table 6.5.
AoSD targeted therapies
| Targeted therapy | International nonproprietary name and usual dose | Reported no. of patients | Preferential AoSD pattern | Follow-up (months) | Complete remission (%) | Steroid dose reduction | References |
|---|---|---|---|---|---|---|---|
| IL-1 receptor antagonists |
Anakinra 100 (sometimes 200) mg/day SC |
>250 | Systemic and articular | >12 | 80 | Yes | [46, 68, 69, 139–144] |
| Anti-IL-1ß |
Canakinumab 150 (sometimes 300) mg/month SC |
<5 | Systemic and articular | >12 | 100 | Yes | [145–147] |
| IL-1 trap |
Rilonacept Loading dose of 100–320 mg and then 100–320 mg/week |
<5 | Systemic and articular | >12 | 100 | Yes | [148] |
| TNF blockers |
Infliximab 3–7.5 mg/kg at W0–W2–W6 and then every 6–8 weeks IV Or Etanercept 50 mg/7 days SC |
<100 | Articular | >12 | 0–100 | n.k. | [149–154] |
| IL-6 receptor antagonists |
Tocilizumab 8 mg/kg/month IV Or 162 mg/week SC |
<150 | Systemic and articular | >12 | 60–85 | Yes | [155–162] |
| Anti-IL-18 |
Tadekinig alfa 80 mg/week SC Or 160 mg/week SC |
<25 | NA | 4 | n.k. | n.k. | [163] |
| B cell directed |
Rituximab 1 g D1–D15 every 6 months |
Single case | NA | 12 | n.k. | n.k. | [164] |
| T cell directed |
Abatacept 500–1000 mg/month IVa Or 125/week SC |
Single case | NA | >12 | n.k. | n.k. | [165, 166] |
D day, IV intravenously, SC subcutaneously, W week, NA not available, IL interleukin, TNF tumor necrosis factor, n.k. not known
aAbatacept dose depends on patient’s weight: <60 kg =500 mg, 60–100 kg = 750 mg, ≥100 kg = 1000 mg