Diagnostic Guidelines of Kawasaki Disease (MCLS: Infantile Acute Febrile Mucocutaneous Lymph Node Syndrome) Adapted from Kawasaki Disease Study Group of the Ministry of Health, Labour and Welfare Japan. The deferencebetween JMC and AHA KD criteria is fever is included in the principal symptoms in JMC and fever is essential to diagnose KD in AHA criteria. Fever plus 3-4/5 criteria score (AHA) is essential to diagnosis of KD and need initial line of treatment such as Intravenous Immunoglobulin and aspirin. 5-6/6 criteria needed if fever is included by JMC The symptoms can be classified into two categories, principal symptoms and other significant symptoms or findings.

Principal symptoms

• Fever persisting for 5 days or more (inclusive of those cases in whom the fever has subsided before the 5th day in response to therapy)
• Bilateral conjunctival congestion
• Changes of lips and oral cavity: Redding of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosa
• Polymorphous exanthema
• Changes of peripheral extremities:
  (Acute phase): Redding of palms and soles, indurative edema
  (Convalescent phase): Membranous desquamation from fingertips.
• Acute nonpurulent cervical lymphadenopathy
  At least five items of 1∼6 should be satisfied for diagnosis of Kawasaki disease.
  However, patients with four items of the principal symptoms can be diagnosed as Kawasaki disease when coronary aneurysm or dilatation is recognized by two-dimensional (2D) echocardiography or coronary angiography.

Other significant symptoms or findings

The following symptoms and findings should be considered in the clinical evaluation of suspected patients.

• Cardiovascular: Auscultation (heart murmur, gallop rhythm, distant heart sounds), ECG changes (prolonged PR/QT intervals, abnormal Q wave, low-voltage QRS complexes, ST-T changes, arrhythmias), chest X-ray findings (cardiomegaly), 2D echo findings (pericardial effusion, coronary aneurysms), aneurysm of peripheral arteries other than coronary (e.g., axillary), angina pectoris or myocardial infarction
• Gastrointestinal (GI) tract: Diarrhea, vomiting, abdominal pain, hydrops of gallbladder, paralytic ileus, mild jaundice, slight increase of serum transaminase.
• Blood: Leukocytosis with shift to the left, thrombocytosis, increased erythrocyte sedimentation rate (ESR), positive C reactive protein (CRP), hypoalbuminemia, increased α2-globulin, slight decrease in erythrocyte and hemoglobin levels.
• Urine: Proteinuria, increase of leukocytes in urine sediment.
• Skin: Redness and crust at the site of BCG inoculation, small pustules, transverse furrows of the finger nails.
• Respiratory: Cough, rhinorrhea, abnormal shadow on chest X-ray.
• Joint: Pain, swelling.
• Neurological: Cerebrospinal fluid (CSF) pleocytosis, convulsion, unconsciousness, facial palsy, paralysis of the extremities.