Study of Combined Therapy IVIG-SN 10% and Acetylsalicylic Acid in Kawasaki Disease

This is a multicenter, single-arm, and open-label clinical trial to evaluate the coronary artery lesions (CALs) at 2 and 7 weeks after single dose of IGIV 10% (2 g/kg) administration for at least 12 hours to evaluate the efficacy and safety of IGIV 10%.

Inclusion criteria

• Infants and children aged 3 months to 7 years inclusive, at the time of signing the informed consent, however, patients with complete KD are eligible up to 12 years (applies to 2A)
• Subjects with fever 4-10 days meeting one of following criteria (A, B or C)

A. Subjects with at least 4 of the following principal clinical findings:

i) Bilateral bulbar conjunctival injection without exudate

ii) Erythema and cracking of lips, strawberry tongue, and/or erythema of oral and pharyngeal mucosa

iii) Erythema and edema of the hands and feet in acute phase and/or periungual desquamation in subacute phase

iv) Cervical lymphadenopathy (≥1.5 cm diameter), usually unilateral

v) Rash: maculopapular, diffuse erythroderma, or erythema multiforme-like

B. Subjects with at least 3 of the principal clinical findings and coronary artery abnormalities detected by 2-dimensional echocardiography: Z score of left anterior descending coronary artery or right coronary artery ≥2.5

C. Subjects without coronary artery abnormalities, but with fever ≥5 days, 2 or 3 of the principal clinical findings , CRP of ≥3.0 mg/dL and meeting 3 or more of the following laboratory findings:

i) Albumin <3.0 g/dL

ii) Anemia for age

iii) Elevated alanine aminotransferase level

iv) Platelet count of >450,000/mm3 after the 7th day of fever

v) White blood cell count of >15,000/mm3

vi) Urine >10 white blood cells/high-power field

• Subjects whose subject's parents or legally authorized representative gave voluntary written consent to participate in the clinical trial

Exclusion criteria

• Subjects with a history of KD
• Subjects with following laboratory findings:

A. Platelet count <100,000/mm3

B. WBC count <3,000 cells/mm3

C. hemoglobin, hematocrit or red blood cell count more than 30% above the upper limit or 30% below the lower limit of the normal range

• Subjects who are currently receiving or who have received any investigational drug or device within 30 days prior to administration of the IP
• Subjects who have received TNF alpha antagonist or systemic corticosteroids within 48 hours prior to administration of the IP
• Subjects who are considered by the investigator to be an unsuitable candidate for the study due to a severe chronic disease (e.g. cardiovascular disease except controllable hypertension, respiratory disease with respiratory failure, metabolic disease, renal failure, hemoglobinopathy, etc.)
• Subjects who have received immunosuppressive or immunomodulatory drugs within 3 months prior to administration of the IP
• Subjects with immunodeficiency including known positive serology for human immunodeficiency virus (HIV)
• Subjects with a history of hypersensitivity or shock to IVIG formulations
• Subjects with underlying liver disease or liver dysfunction with known etiology
• Subjects with renal impairment whose creatinine level is more than twice the upper limit of the normal range
• Subjects with a history of malignant tumor
• Subjects with a history of IgA deficiency
• Subjects who are considered by the investigator to be an unsuitable candidate for the study for any reason