Table 4 Other drugs used in the drug-resistant Kawasaki disease

Mechanism of action

Inhibitor of human trypsin

Inhibition of calcineurin and increased activity of T cells

Folic acid antagonist, suppression of lymphoproliferation

Indications

Patients resistant to IVIG

(also as initial treatment combined with IVIG and ASA in high-risk patients)

Patients resistant to IVIG

(there are no studies in patients < 4 months of life)

Patients resistant to IVIG

Dosage

Optimal dosing in not yet determined in children, though in many studies

The dosage is 5000 U/kg for 3–6 times per day (maximum dose: 50000 U); it should be given in a second vein or temporarily suspending IVIG infusion (as it becomes turbid in contact with other drugs)

4 mg/kg/day in 2 doses per os; in case of persistence of fever the dosage can be increased to 5–8 mg/kg/day; administered until CRP normalization or for 10–14 days

10 mg/m²/week per os, administered until fever disappears

Side effects

Anaphylactic shock, liver dysfunction, leukopenia, rash, itching, diarrhea, pain at the injection site

Hypercalcemia, hypomagnesemia, hirsutism, hypertension

Gastrointestinal signs, alopecia, risk of myelosuppression, anaphylaxis, infections, liver dysfunction, acute kidney failure

Level of evidence

Class III, grade C

(First-line treatment with IVIG + ASA: class II, grade B)

Class V, grade C

Class V, grade C