Drugs | Dose | Remarks | Reference |
1. IVIG | 2 g/kg 8 - 10 hours, single infusion | Recommended treatment in IVIG resistant KD. | [5] |
2. IVIG + Corticosteroid | Methylprednisolone 30 mg/kg per day for up to 3 days or prednisolone 1 - 2 mg/kg/day for at least 15 days | Commonly used alternative treatment. | [5] |
3. Anti (TNF) - alpha agent |
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- Infliximab | 5 mg/kg IV 2 hours | Progression of CAAs was similar in both groups. These agents can rapidly normalize laboratory markers of inflammation. | [30] |
- IVIG + Infliximab | IVIG + Infliximab 5 mg/kg IV | ||
- Etanercept | 0.8 mg/kg 3 doses | Still in research phase for IVIG resistant. Trial of etanercept as adjuvant with IVIG in initial therapy showed no decrease in IVIG resistant. | [31] |
4. Interleukin-1 (IL-1) Inhibition |
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- anakinra - canakinumab | 2 - 6 mg/kg/day by s/c injection - | Are in trial phase. Initial reports suggesting these drugs are safe to children but data regarding their efficacy is still not available. | [32] [33] |
5. Plasmapheresis |
Plasma exchange | Complex and suggested to use in children with all other failed pharmacologic treatment. Showed benefit in some small sized studies. Not applicable. | [34] [35] [36] |
6. Other Immunosuppressive Agents |
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- cyclophosphamide - cyclosporine - Tocilizumab | - - - | Studies been carried out in a very limited numbers of patients and centers. Studies are still going on these drugs but due to its toxicity these are not front lines drugs for refractory KD. | [37] [38] [1] |
- Methotrexate | [10 mg/body surface area (BSA)] till fever subside | Showed improvement in reduction of symptoms but no in improvement or prevention of CAL | [39] |