• Clinical science

Kawasaki disease (Mucocutaneous lymph node syndrome)

Abstract

Kawasaki disease is an acute, necrotizing vasculitis of unknown etiology. The condition primarily affects children under the age of five and is more common among those of Asian descent. The disease is characterized by a high fever, desquamative rash, conjunctivitis, mucositis (e.g., “strawberry tongue”), cervical lymphadenopathy, as well as erythema and edema of the distal extremities. However, coronary artery aneurysms are the most concerning possible manifestation as they can lead to myocardial infarction or arrhythmias. Kawasaki disease is a clinical diagnosis, further supported by findings such as elevated ESR or evidence of cardiac involvement in echocardiography. Treatment with IV immunoglobulins and high-dose aspirin is essential and should be initiated immediately after diagnosis.

Epidemiology

  • Sex: > (1.5:1)
  • Age: primarily children < 5 years
  • Peak incidence: occurs mostly in late winter and spring
  • Prevalence: : approx. 20 per 100,000 children; highest rate in children of Asian and Pacific-Islander descent
  • Mortality: approx. 1%

References:[1][2][3][4]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

The exact cause of Kawasaki disease remains uncertain. However, it is associated with infectious and genetic factors.

References:[1][3]

Clinical features

  • Clinical diagnosis requires fever for at least 5 days and:
    • ≥ 4 other specific symptoms, or
    • < 4 specific symptoms if the coronary arteries are involved
  • Specific symptoms
    • Erythema and edema of hands and feet, including the palms and soles (the first week)
      • Possible desquamation of fingertips and toes (after 2–3 weeks)
    • Polymorphous rash, originating on the trunk
    • Painless bilateral “injected” conjunctivitis without exudate
    • Oropharyngeal mucositis
    • Cervical lymphadenopathy (mostly unilateral)
  • Nonspecific symptoms may precede the onset of Kawasaki disease (e.g., diarrhea, fatigue, abdominal pain)

Always consider Kawasaki disease in small children with a rash and high fever unresponsive to antibiotics!

References:[1][2]

Diagnostics

Kawasaki disease is a clinical diagnosis (see “Clinical features” above) which is supported by the following diagnostic findings:

References:[1]

Treatment

  • IV immunoglobulin (IVIG)
    • high single-dose to reduce the risk of coronary artery aneurysms
    • most effective if given within the first 10 days following disease onset
  • High-dose oral aspirin
  • IV glucocorticoids: may be considered in addition to standard treatment in high-risk patients , as they lower the risk of coronary involvement; also in cases of treatment-refractory disease

To avoid the risk of Reye syndrome, children should not be treated with aspirin, especially if a viral infection is suspected. Kawasaki disease is an exception to this rule!References:[1][4]

Complications

References:[4]

We list the most important complications. The selection is not exhaustive.