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

- 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

- 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

- 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]