| Initial immunomodulatory therapy (first-line treatment) |
| IVIG | 2 gm/kg over 12 hours(based on ideal body weight, maximum 100 gm) | Patients with depressed cardiac function require close monitoring and diureticsIn patients with cardiac dysfunction, IVIG may be given in divided doses (1 gm/kg daily over 2 days) |
| Low-to-moderate dose glucocorticoid | MPD 1–2 mg/kg/day | |
| Intensification immunomodulatory therapy (for refractory disease) |
| High-dose glucocorticoid | MPD 10–30 mg/kg for 1–3 days | In patients who require high-dose or multiple inotropesSubsequent return to low-to-moderate dosing |
| Anakinra | 5–10 mg/kg/day IV or SC daily | In patients with features of MAS or with contraindications to long-term use of glucocorticoidsUsed in some patients for a long period (up to 2 weeks) as a steroid-sparing agent |
| Infliximab | 5–10 mg/kg/day IV or SC for 1 dose | Alternative biological agent to anakinraShould not be used to treat patients with features of MAS |
| Antiplatelet therapy |
| Low-dose aspirin | 3–5 mg/kg/day | Should be continued until the platelet count is normalized and normal coronary arteries are confirmed at ≥ 4 weeks after diagnosis |
| Anti-coagulation therapy Enoxaparin | Anti-factor Xa level 0.5–1.0 | For coronary arterial aneurysm (z-score ≥ 10.0) or decreased EF (< 35 %)Initial anticoagulation therapy for at least 2 weeks |
| Warfarin | INR 2–3 | Can be used after initial anticoagulation using enoxaparin |