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

From: Kawasaki disease: guidelines of Italian Society of Pediatrics, part II - treatment of resistant forms and cardiovascular complications, follow-up, lifestyle and prevention of cardiovascular risks

  Ulinastatin [93, 94] Cyclosporine A [95,96,97] Methotrexate [98]
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