3. Median age of affected children = 2.3 years
80% of cases in children < 4 yrs,
5% of cases in children > 10 yrs
Males 1.7 : 1 Females
Positive family history in 1% but 13% risk of
occurrence in twins.
Epidemiology
4. Annual incidence: 4-15/100,000 children under 5
years of age
Japanese, Chinese, Korean 10-15 time more than
others
Seasonal variation: more cases in winter and spring
but occurs throughout the year
5. No causative agent identified, but it could be:
Bacterial, retroviral, superantigenic bacterial toxin
Infectious agent features:
Age-restricted susceptible population
Seasonal variation
Acute self-limited illness
Etiology
6. Identified a novel human coronavirus in respiratory
secretions from a 6-month-old with typical Kawasaki
Disease
Subsequently isolated from 8/11 (72.7%) of Kawasaki
patients & 1/22 (4.5%) matched controls (p = 0.0015)
Suggests association between viral infection &
Kawasaki disease
7. Fever (39-40°) for at least 5 days
(no response to Paracetamol or others NSAIDs)
At least 4 of the following 5 features:
1. Conjunctival injection
2. Erythema or fissuring of lips and oral cavity:
strawberry tongue
3. Cervical lymphadenopathy
(the least common among those symptoms 60% vs 90%)
Polymorphous exanthem, usually truncal
Changes in the extremities:
Edema, erythema,
Diagnostic Criteria
8. Present with < 4 out of 5 diagnostic criteria
Compatible laboratory findings
Still develop coronary artery aneurysms
(children with incomplete KD are also at risk for
cardiovascular sequelae)
No other explanation for the illness
Incomplete (Atypical) Kawasaki Disease
9. Acute (1-2 weeks from onset)
Febrile (39-40°C), irritable
Oral changes, rash, edema/erythema of feet
Desquamation, arthritis or arthralgias
Subacute (2-8 weeks from onset)
Temperature decrease
Hypercoagulability (> PLT)
High risk coronaries artery vasculitis and aneurysm
Gradual improvement even without treatment
Convalescent (Months to years later)
Phase of the Disease
22. Myocarditis
Present in 50% of the patients
Tachycardia, murmur, gallop rhythms
Disproportionate to degree of fever & anemia
ECG changes
Prolonged PR and/or QT intervals
Low voltage (especially of the R wave)
ST-T–wave changes.
Cardiovascular Manifestation
of Acute kawasaki disease
23. Pericarditis
Present in 25% although symptoms are rare
Distant heart tones, pericardial friction rub,
tamponade
Valves abnormalities
Valves incompetence in 1 % patients
24. Coronary Arterial changes
15% to 25 % of untreated patients develop
coronary artery changes
3 - 7% if treated in first 10 days of
fever with IVIG
Most commonly proximal, can be distal
Left main > LAD > Right
May lead to myocardial infarction,
sudden death, or ischemic heart disease
25. Risk Factors
Younger than 6 months, older than 8 years
Males
Fevers persist for greater than 14 days
Persistently elevated ESR
Thrombocytosis
Pts who manifest s/s of cardiac involvement
26. Size
Small = <5 mm diameter
Medium = 5-8 mm
Giant = ≥ 8 mm
Shape
Saccular
Fusiform
27. Prognosis:
about 50% of aneurysms resolve, it depends
on:
Smaller size= <5 mm diameter
Fusiform Morphology
Female gender
while, the giant aneurysms have the worst prognosis
Highest risk for sequelae
Other arteries may be involved:
renal
cerebral arteries
peripheral arteries (upper and lower extremities)
28. If the echocardiogram is negative but fever persists, a
repeat echocardiogram may be performed.
If the echocardiogram is negative and the
fever abates, Kawasaki disease is unlikely.
If the echocardiogram is
positive, the child is treated for Kawasaki disease.
Myocarditis with dysfunction
Pericarditis with an effusion
Valvar insufficiency
Coronary arterial changes
Echocardiographic findings
29.
30. 0.3-2% mortality rate due to cardiac disease
10% from early myocarditis
32% from MI: principal cause of death in KD
Aneurysms may thrombose, leading to MI/death
Most often in the first year
Majority while at rest/sleeping
About 1/3 asymptomatic
Cardiovascular Sequelae
31. IVIG: 2g/kg as one-time dose
Mechanism of action is unclear
Significant reduction in Coronary arterial
Aneurysm in pts treated with IVIG plus aspirin
vs. aspirin alone (15-25%3-5%)
70-90% defervesce and resolution within 2-3
days of treatment
Treatment
32. Aspirin
High dose (80-100 mg/kg/day) for its
antiinflammatory effects (e.g., reduce the
duration of fever), until afebrile x 48 hrs
(need high doses in acute phase due to
malabsorption of ASA)
NOTE
Dosage of ASA in acute phase does not seem to
affect subsequent incidence of CAA
33. Decrease to low dose (3-5 mg/kg/day), for its
antiplatelet activity, for 6-8 weeks or until platelet
levels normalize.
NOTE: for patients who have aneurysms, aspirin
should be continued until the aneurysm resolves
or should be continued indefinitely.
No evidence of effect on CAA when used alone.
Due to potential risk of Reye syndrome instruct parents
about symptoms of influenza or varicella
34. Aggressive support with diuretics &
inotropes
for some patients with
myocarditis
Antibiotics (while excluding bacterial
infection)
A good place to start is to define the disease. This quote is a as good a definition as I’ve found.
Kawasaki disease is a self-limited vasculitis of unknown etiology that predominantly affects children younger than 5 years. It is now the most common cause of acquired heart disease in children in the United States and Japan.
Another important point to make is that, in part due to the fact that we have no etiology, there is no specific diagnostic test for Kawasaki disease.
For this reason, some people call it Kawasaki syndrome and not Kawasaki disease.
Therefore, guideline have been developed to make the diagnosis.
But, the diagnosis of Kawasaki disease is still one of exclusion.
Kawasaki disease is virtually unheard of in children older than 15 years of age.
A number of each of these has been implicated in individual cases or clinical series but typically data are conflicting.
A variant strain of Propionibacterium acnes with dust mites playing a role as vectors has been proposed as the causative agent by several investigators but no causative link has been established despite multiple investigations.
The diagnosis of Kawasaki Disease requires fever persisting at least 5 days (although US and Japanese experts now agree that only 4 days of treatment are necessary before initiating treatment) and the presence of at least 4 of the following 5 principal features: changes in extremities, a polymorphous rash, conjunctival injection, changes in lips and oral cavity, and cervical lymphadenopathy.
In addition, other diagnoses must also be ruled out.
For example scarlet fever, toxic shock syndrome, and adenoviral infections have similar presentation.
The fever is usually high, greater than 102°F, does not respond well to antipyretics, and can last 1-2 weeks.
The important thing to remember though is that Kawasaki disease is a diagnosis of exclusion without confirmatory tests s and based on a constellation of signs and symptoms and/or consistent ultrasound findings
In this convalescent phase remaining symptoms resolve & laboratory values normalize.
Acute changes seen in the hands and feet are usually seen several days into the course.
There is erythema and edema of hands and feet, usually on the dorsal surfaces.
Children may also refuse to walk or have trouble putting on their shoes.
Erythema of palms & soles &/or painful edema of hands/feet
Usually start 3-5 days after onset of fevers
Subungual desquamation in subacute phase
Beau lines may be seen 2-3 months after onset
Acute changes seen in the hands and feet are usually seen several days into the course.
There is erythema and edema of hands and feet, usually on the dorsal surfaces.
Children may also refuse to walk or have trouble putting on their shoes.
Erythema of palms & soles &/or painful edema of hands/feet
Usually start 3-5 days after onset of fevers
Subungual desquamation in subacute phase
Beau lines may be seen 2-3 months after onset
Acute changes seen in the hands and feet are usually seen several days into the course.
There is erythema and edema of hands and feet, usually on the dorsal surfaces.
Children may also refuse to walk or have trouble putting on their shoes.
Erythema of palms & soles &/or painful edema of hands/feet
Usually start 3-5 days after onset of fevers
Subungual desquamation in subacute phase
Beau lines may be seen 2-3 months after onset
Acute changes seen in the hands and feet are usually seen several days into the course.
There is erythema and edema of hands and feet, usually on the dorsal surfaces.
Children may also refuse to walk or have trouble putting on their shoes.
Erythema of palms & soles &/or painful edema of hands/feet
Usually start 3-5 days after onset of fevers
Subungual desquamation in subacute phase
Beau lines may be seen 2-3 months after onset
Acute changes seen in the hands and feet are usually seen several days into the course.
There is erythema and edema of hands and feet, usually on the dorsal surfaces.
Children may also refuse to walk or have trouble putting on their shoes.
Erythema of palms & soles &/or painful edema of hands/feet
Usually start 3-5 days after onset of fevers
Subungual desquamation in subacute phase
Beau lines may be seen 2-3 months after onset
Acute changes seen in the hands and feet are usually seen several days into the course.
There is erythema and edema of hands and feet, usually on the dorsal surfaces.
Children may also refuse to walk or have trouble putting on their shoes.
Erythema of palms & soles &/or painful edema of hands/feet
Usually start 3-5 days after onset of fevers
Subungual desquamation in subacute phase
Beau lines may be seen 2-3 months after onset
Acute changes seen in the hands and feet are usually seen several days into the course.
There is erythema and edema of hands and feet, usually on the dorsal surfaces.
Children may also refuse to walk or have trouble putting on their shoes.
Erythema of palms & soles &/or painful edema of hands/feet
Usually start 3-5 days after onset of fevers
Subungual desquamation in subacute phase
Beau lines may be seen 2-3 months after onset
Acute changes seen in the hands and feet are usually seen several days into the course.
There is erythema and edema of hands and feet, usually on the dorsal surfaces.
Children may also refuse to walk or have trouble putting on their shoes.
Erythema of palms & soles &/or painful edema of hands/feet
Usually start 3-5 days after onset of fevers
Subungual desquamation in subacute phase
Beau lines may be seen 2-3 months after onset
The complete list of associated symptom is too long to go into in detail here, but virtually every organ system can be involved.
Patients can have cough, rhinorrhea, or a pulmonary infiltrate.
They can have diarrhea, vomiting, abdominal pain, hydrops of gallbladder, mild jaundice, and mild increase of serum transaminase levels.
They can have striking irritability and an aseptic meningitis with a mononuclear pleocytosis in cerebrospinal fluid as well as a facial palsy and hearing loss.
Finally, they can develop myositis, arthralgias, and arthritis.
Heme: hemophagocytosis
Renal: ARF, renal artery aneurysms
Skin: transverse furrows of fingernails (Beau’s lines) during convalescence
Others: Peripheral gangrene, orbital myositis, Avascular necrosis of the femoral head
In fact, these 2 etiologies have been found to account for over 80% of patients initially thought to have Kawasaki disease but ultimately not diagnosed as such.
These would include toxic shock syndrome & staph scalded skin syndrome.
In acute phase, the most common blood laboratory findings are: a leukocytosis with left shift, mild anemia, an increased erythrocyte sedimentation rate or C-reactive protein, hypoalbuminemia, elevation of liver transaminases
Later in the illness, the platelet count tends to rise during the second week and can remain elevated for longer than 6-8 weeks.
Finally, the urine can show sterile pyuria of urethral origin and occasional proteinuria.
From the cardiovascular standpoint,
Patients can have no signs or symptoms.
Sometimes, they have tachycardia out of proportion to the extent of fever.
They can have a gallop rhythm or distant heart sounds suggestive of myocarditis or pericarditis with an effusion.
They can have a flow murmur or a murmur due to valvar insufficiency.
Or they can have frank congestive heart failure and/or an infarction.
Their EKG can show a number of changes, including arrhythmias, abnormal Q waves, prolonged PR and/or QT intervals, low voltage, and ST-T–wave changes.
Their chest x-ray can show cardiomegaly or pulmonary edema.
The murmur may be due to valvar insufficiency or to flow in a high output state.
Ectasia is defined as coronary artery size larger than normal for age but without discrete aneurysm.
Ectasia is defined as coronary artery size larger than normal for age but without discrete aneurysm.
Ectasia is defined as coronary artery size larger than normal for age but without discrete aneurysm.
Ectasia is defined as coronary artery size larger than normal for age but without discrete aneurysm.
Echocardiographic finding include myocarditis and depressed function, pericarditis with an effusion, valvular insufficiency, or coronary artery changes.
The most common finding is mild coronary artery changes.
Echo is 80-90% sensitive for the diagnosis of proximal coronary artery aneurysms
Angiography is the gold standard and can define proximal and distal coronary anatomy better than echocardiography.
However, performing angiography on all patients is unreasonable.
In addition, angiography may miss abnormalities in the arterial wall, including thromboses or dysfunction.
Rarely, aneurysms may rupture.
Typically given as this one time dose although different regimens have been used including 500/kg/day x 4 days.
…and actually it was tried after the publication of successful IVIG therapy of ITP in 1981. It was endorsed as recommended therapy by the Committee on Infectious Diseases of the AAP in 1988.
A multicenter prospective trial in the US has demonstrated …
In addition to fewer giant coronary aneurysms children receiving IVIG also had more rapid resolution of abnormalities of myocardial function.
Despite this, it’s typically recommended that children diagnosed after 10 days still be treated if there is evidence of continuing inflammation or evolving coronary artery disease.
Otherwise the platelet level can be repeated a month later .
By 6-8 weeks if the coronaries continue to remain normal appearing it is extremely unlikely that they will develop any any any late changes.
