Patient hemodynamically stable without severe features |
Patient with hypotensive shock, respiratory failure, severe neurologic involvement |
First-line treatment |
First-line treatment |
IVIG 2 g/kg |
IVIG 2 g/kg + methylprednisolone 1–2 mg/kg/day |
Monitor for improvement in fever curve, inflammatory markers |
If life or organ threatening disease, consider methylprednisolone 10–30 mg/kg/day |
Second-line treatment for refractory disease |
Second-line treatment for refractory disease |
Addition of methylprednisolone 1–2 mg/kg/day |
Intensify steroid regimen to methylprednisolone 10–30 mg/kg/day |
Consider alternative second-line agent (anakinra, infliximab, etc.) |
Addition of high-dose anakinra or alternative second-line agent |
Additional medications |
Initiate anti-platelet therapy with aspirin 3–5 mg/kg daily (max. 81 mg/day) |
Initiate anticoagulation vs anti-platelet therapy; hold anti-platelet therapy for significant thrombocytopenia |
If on steroids and aspirin, initiate gastric prophylactic medication |
If on high-dose steroids ± aspirin, initiate gastric prophylactic medication |