Table 2.
Agent | Dosing and regimen | Special considerations |
---|---|---|
IVIG |
2 gm/kg/dose Maximum 70–100 g/dose |
IVIG dosing is based on ideal body weight In patients with cardiac dysfunction, IVIG may be given in divided doses of 1 gm/kg daily over 2 days |
Glucocorticoids Prednisone Prednisolone Methylprednisolone |
Mild disease: 1–2 mg/kg/day Moderate disease: 10 mg/kg/day Severe disease: 30 mg/kg/day Maximum 1000 mg/dose |
Add low-to-moderate dose glucocorticoids to IVIG for patients with shock and/or organ-threatening disease Add high-dose, IV glucocorticoids for patients refractory to IVIG and low-to-moderate dose glucocorticoids and/or requiring multiple inotropes and/or vasopressors |
Anakinra | > 4 mg/kg/day IV or SC | Consider in patients with disease refractory to IVIG and glucocorticoids, features of macrophage activation syndrome, shock requiring multiple inotropes and/or vasopressors, and/or contraindications to long-term use of glucocorticoids |
Tocilizumab |
< 30 kg, 12 mg/kg/dose IV ≥ 30 kg, 8 mg/kg/dose IV Maximum 800 mg/dose |
Limited evidence for use based largely on case reports and case series. Considerations for use are similar to anakinra and availability may vary across centers |