Kawasaki Disease

Kawasaki Disease

Diagnostic criteria, clinical findings, diagnostic tests & treatments

  • Kawasaki Disease is the leading cause of heart disease in children with a high incidence in children of Asian ancestry
  • The majority of cases occur in children < 5 years of age
  • Cardiac problems are major cause & concern of the disease
  • CBC- (WBC ↑), ESR ↑, C-reactive protein ↑
  • Echocardiogram should be ordered in acute phase as well as follow up echocardiograms
  • Treatment is IV gamma globulin and Aspirin
  • Kawasaki disease is not contagious
  • Without treatment, duration of fever is about 12 days
  • Complications & symptoms of Kawasaki disease should resolve in 6-8 weeks following treatment, but myocardial infarction & sudden death can occur months to years later
  • Coronary aneurysms occur in 20-25% of untreated patients
  • Carditis may involve the pericardium, myocardium & endocardium
  • Mitral & aortic regurgitation may develop
  • Myocardial infarction is the major cause of death for patients with Kawasaki disease

Diagnostic Criteria

  • Fever is the prominent symptom, lasting 1-2 weeks or longer despite use of antibiotics & antipyretics
  • Fever at least for 5 days with 4 of the 5 symptoms:
  • ♦Changes in extremities (erythema & edema in hands & feet, desquamation of fingertips)
  • ♦Polymorphous exanthema (rash)
  • ♦Bilateral painless conjunctival injection without exudate (red eyes)
  • ♦Changes in lips & oral cavity (strawberry tongue, erythema & cracking of lips)
  • ♦Cervical lymphadenopathy (usually unilateral & involving at least 1 node ≥ 1.5 cm in diameter)

PEEL (Menmonic)

  • P=Polymorphous exanthema
  • E=Extremities (hand & feet erythema)
  • E=Eyes (conjunctival injection)
  • L=Lymphadenopathy (cervical lymphadenopathy of at least 1 node)

Clinical Findings

  • Perineal rash & desquamation in subacute phase
  • During convalescence, fingernails may show furrows or Beau’s lines
  • Respiratory: cough, rhinorrhea, and pulmonary infiltrate
  • Gastrointestinal: diarrhea, vomiting, and pain, hydrous of gallbladder, paralytic ileum, and mild jaundice
  • Joints: arthralgia & arthritis
  • Neurological: irritability & facial palsy (rare)


  • Do complete history
  • Assess for dehydration
  • Measure vital signs
  • Inspect skin
  • Preform complete eye exam
  • Assess ear, nose, throat
  • Assess neck for lymphadenopathy & nuchal rigidity
  • Perform complete cardiovascular exam
  • Auscultate lungs
  • Palpate abdomen for organomegaly & tenderness
  • Assess joints for edema, erythema, and tenderness
  • Preform a neurological exam
  • Assess for testicular edema in males

Diagnostic Tests

No specific diagnostic tests are available but order:

  • CBC (leukocytosis with left shift)
  • Erythrocyte sedimentation rate (ESR) – usually elevated
  • C-reactive protein- usually elevated
  • Urinalysis (pyuria & sometimes proteinuria)
  • Serum transaminases- usually elevated
  • Albumin (decreased in acute phase)
  • Lumbar puncture (mononuclear pleocytosis in CSF)
  • Echocardiogram
  • Coronary angiography is considered by some as the “gold standard” to assess vascular artery disease but has risks
  • Consult specialist & arrange for hospitalization for patients with suspected Kawasaki disease
  • Patient should be managed by a cardiologist

Differential Diagnosis

  • Rubeola
  • Scarlet fever
  • Lyme disease
  • Rocky Mountain spotted fever
  • Infectious mononucleosis
  • Roseola
  • Drug reactions (Stevens-Johnson syndrome)
  • Scalded skin syndrome
  • Toxic shock syndrome
  • Juvenile rheumatoid arthritis
  • Mercury poisoning
  • Anthrax
  • Smallpox
  • Hemorrhagic fever (caused by Ebola or Marburg viruses)


Treatment is supportive care & anti-infammatory therapy

  • Refer- consult cardiologist & arrange for hospitalization
  • Treatment should begin immediately: treat with intravenous gamma globulin & aspirin for the first 10 days
  • Treatment on or before 5 days of fever may prevent coronary inflammation and damage to arteries
  • Instruct parents to notify health care provider immediately if child is suspected of having influenza or varicella (↑risk of developing Reye’s syndrome from taking aspirin)
  • Instruct parents on signs & symptoms of possible complications: arthralgia,chest pain, & palpitations

Subsequent Immunizations

  • Do not administer varicella & measles vaccines for 11 months after gamma globulin therapy
  • May administer other scheduled immunizations
  • Administer influenza vaccine yearly to patients 6 months-18 years of age

Follow Up

  • Patients should be examined frequently during the first 2 months to detect arrhythmias, heart failure, valvular problems, & myocarditis
  • Echocardiogram should be ordered in acute phase followed by subsequent echocardiograms at 3-8 weeks after onset of illness
  • Additional echocardiograms will depend on degree of coronary artery involvement
  • Patients should be managed by cardiologist

Pictures of Kawasaki Disease