KAWASAKI

DISEASE
AGENDA
Etiology & Epidemiology
Clinical Manifestations
Laboratory and Imaging Studies
Diagnosis
Treatment
Complications & Prognosis
Kawasaki Disease 3

ETIOLOGY &
EPIDEMIOLOGY
Kawasaki disease is a vasculitis. It has unknown etiology and is characterized by
multisystem involvement and inflammation of small to medium sized arteries. This
can result in aneurysm formation.

Kawasaki disease is more common in children of Asian descent. It is the second most
common vasculitis of childhood. The highest frequency of Kawasaki disease is found
in Japan. It is most common in males under five years of age. It peaks at age 2-3, and
it is rare in children older than 7. In the United States, this disease affects
approximately 6 per 100,000 children under 5 years of age and is the leading cause of
acquired heart disease.

CLINICAL MANIFESTATIONS
ACUTE PHASE
1-2 weeks
Sudden onset of a high fever. After the
fever, conjunctival erythema, cracked
lips, strawberry tongue, cervical
lymphadenopathy, and swelling of the
hands and feet may occur. Conjunctivitis
is often bilateral. Cervical
lymphadenopathy >1.5cm is found in
70% of children during this phase. Giant
coronary artery aneurysms may occur.
4
SUBACUTE CONVALESCENT
PHASE PHASE
Until week 4 6-8 weeks
Gradual resolution of fever and other Disappearance of clinical
symptoms. Desquamation of the skin may symptoms. Concludes when the
occur during this phase, particularly of the ESR returns to normal. Linear
fingers and toes. Coronary artery aneurysms nail creases may appear.
may occur during this phase. Risk factors for
the development of coronary artery
aneurysms include prolonged fever, age under
1 or over 6, and male gender.
Kawasaki Disease 5

CRASH & BURN

C R A S H
CONJUNCTIVITIS RASH ADENOPATHY STRAWBERRY HANDS AND
TONGUE FEET
SWELLING

BURN = HIGH FEVER

Kawasaki Disease 6

LABORATORY AND
IMAGING STUDIES
Blood and urine cultures are important to obtain to rule out other causes of fever. It is
important to ensure that there is not an infectious cause of the patient’s fever or rash.
The chest x-ray can also rule out infectious causes. In the acute phase of Kawasaki
disease, white blood cell count, C-reactive protein, and ESR are often elevated. The
ESR elevation can be profound, often greater than 80 mm (normal is less than 20
mm). Greatly elevated platelet counts can develop during the subacute phase.
Coronary artery aneurysm development is monitored by echocardiogram.
Echocardiograms are usually performed during the acute phase, at 1 to 2 weeks, and
again at 4 to 6 weeks from symptom onset.
Kawasaki Disease 7

DIAGNOSIS
Kawasaki disease is diagnosed based on the presence of fever at least four of the five
following criteria:
o Bilateral nonsuppurative conjunctivitis
o One or more changes of the mucus membranes, including strawberry tongue
o One or more changes of the extremities, including desquamating rash
o Polymorphous rash, primarily on the trunk
o Posterior cervical lymphadenopathy greater than 1.5 cm in diameter
o Disease cannot be explained by another known disease process
Kawasaki Disease 8

TREATMENT
Kawasaki disease is treated with intravenous immunoglobulin. A single dose of
intravenous immunoglobulin (2 g per kilogram over 12 hours) rapidly causes a
resolution of clinical illness. Aspirin is given in anti-inflammatory doses (60 to 80 mg
per kilogram per day divided every six hours) in the acute phase. When the fever
resolves, aspirin is reduced to anti-thrombotic doses (3 to 5 mg per kilogram per day
as a single dose) and is given for 4 to 6 weeks until follow up echocardiogram
documents the absence or resolution of coronary artery aneurysms.
If one dose of intravenous immunoglobulin is not effective, another dose may be
given. If that is still not effective, corticosteroids or infliximab may be used.
Kawasaki Disease 9

COMPLICATIONS
AND PROGNOSIS
Most cases of Kawasaki disease resolve without complications. Myocardial infarction
has been noted in children who have had Kawasaki Disease in the past, likely due to
stenosis of a coronary artery at the site of an aneurysm.

The prevalence of coronary artery disease in children with Kawasaki Disease is

decreased from 20 to 25% in children treated with aspirin alone to less than 5% in
children treated with aspirin and intravenous immunoglobulin. Inflammation of
duration greater than 10 days increases the patient’s risk for developing coronary
artery aneurysms.
Kawasaki Disease 10

REFERENCES
Behrman RE. Kawasaki Disease (Laryngotracheobronchitis) In: Nelson
Textbook of Pediatrics / Richard E. Behrman [and Three Others]
Editors. Twenty-one edition. (Behrman RE, ed.). Elsevier; 2023
THANK YOU