Kawasaki disease Dr. Abdelqader wishah

INTRODUCTION
•
Kawasaki disease (KD, previously called mucocutaneous lymph node
syndrome) is one of the most common vasculitis of childhood after
(Henoch-Schönlein purpura) , particularly in East Asia. It is typically a self-
limited condition, with fever and other acute inflammatory manifestations
lasting for an average of 12 days if not treated. The underlying etiology is
unknown.

Epidemiology
•
Japan :215 per 100,000 per year
•
United States :20 per 100,000 children
•
Occupied Palestine : 7 per 100,000 in 2000 to 2004

Risk factors
•
geographic and ethnic variation
•
Boys are affected as much as 50 percent more commonly than
girls
•
younger than five years
•
Rare beyond late childhood
•
uncommon among children younger than six months
•
More in winter/spring

CLINICAL MANIFESTATIONS
•
The clinical features of KD reflect widespread inflammation of
primarily medium-sized muscular arteries. Diagnosis is based upon
evidence of systemic inflammation (eg, fever) in association with
signs of mucocutaneous inflammation.
•
findings are often not present at the same time, and there is no
typical order of appearance.

Fever
•
An elevated body temperature is the most consistent manifestation
of KD. Fever is minimally responsive to antipyretic agents, and it
typically remains above 38.5ºC (101.3ºF) during most of the illness.
On the other hand, fever may be intermittent and may be missed by
parents

In 90 percent of patients and characteristically spares the limbus

polymorphous. The rash usually begins during the first few
days of illness, typically as perineal erythema and
desquamation

least consistent feature of KD, absent in as many as one-half
to three-quarters of children primarily involve the anterior
cervical nodes overlying the sternocleidomastoid muscles.
Often, only a single, large node is palpable, although ultrasound
imaging of the neck typically reveals numerous discrete nodes
arranged like a bunch of grapes

Arthritis
•
7.5 to 25 percent of patients with KD
•
large joints (ie, knee, ankle, and hip) were primarily involved.

Cardiovascular findings
•
Tachycardia out of proportion to the degree of fever and gallop sounds.
These physical exam findings are the result of lymphocytic myocarditis that
is ubiquitous in children with KD. In addition, heart sounds may be muffled
due to a pericardial effusion, which is detected in approximately 30 percent
of children
•
MURMER
•
ECG :Arrythmias ,prolonged pr/qt interval
•
CXR:Cardiomegaly

echo
•
30 percent of patients with KD are found to have CA dilatation at
diagnosis .
•
Frank aneurysms are usually not seen until after day 10 of illness
•
fusiform aneurysms of other nonvisceral medium-sized arteries, most
characteristically involving the brachial arteries.
•
should be performed in all patients with KD as soon as the
diagnosis is suspected
•
Follow-up studies — Repeat echocardiograms are usually obtained at
one to two weeks and again four to six weeks after discharge.
•
Myocarditis /pericarditis .

Other findings
•
nonspecific symptoms commonly occur during the prodrome of the
illness, 7 to 10 days before the typical mucocutaneous features.
•
Diarrhea, vomiting, or abdominal pain – 61 percent
Irritability – 50 percent (older children with KD more commonly present with
lethargy than irritability)
•
Vomiting alone – 44 percent
•
Cough or rhinorrhea – 35 percent

•
Decreased oral intake – 37 percent
•
Joint pain – 15 percent
•
NB: Patients with gastrointestinal involvement often have pseudo-
obstruction on radiologic studies

•
NB: Treatment with intravenous immune globulin (IVIG) usually
raises the ESR, so this lab test should not be measured after a
child receives IVIG. On the other hand, control of inflammation by
IVIG accelerates the decrease in CRP
.
•
Lymphocyte numbers typically drop during the acute phase of KD,
then rise dramatically during convalescence.
•
Platelet counts generally rise by the second week of illness and
may reach 1,000,000/mm3

CSF
•
may display a mononuclear pleocytosis without hypoglycorrhachia
(decreased CSF glucose) or elevation of CSF protein.
•
Similarly, arthrocentesis of inflamed joints in KD typically
demonstrates a pleocytosis, with 125,000 to 300,000 WBCs/mm3,
primarily neutrophils

•
Incomplete KD should be suspected in patients less than six
months of age with unexplained fever ≥7 days, even if they have no
clinical findings of KD, and in patients of any age with unexplained
fever ≥5 days and only two or three clinical criteria.

DIFFERENTIAL DIAGNOSIS
•
KD is most commonly confused with infectious exanthems of
childhood Early in the course, KD is often mistaken for more routine
childhood illnesses, such as viral gastroenteritis, viral upper
respiratory tract infection, or pneumonia, depending upon the other
presenting symptoms
•
NB:concurrent infections (both viral and bacterial) are common in
patients with KD, found in up to 33 percent of children

•
Infectious diseases and other mimics of KD may have the following clinical
features not commonly found in KD :
•
●Exudative conjunctivitis (eg, adenovirus)
•
●Exudative pharyngitis (eg, streptococcal pharyngitis)
•
●Discrete intraoral lesions (eg, Koplik spots in measles)
•
●Bullous or vesicular rash (eg, Stevens-Johnson syndrome [SJS])
•
●Generalized lymphadenopathy (eg, Epstein-Barr virus [EBV] infection)
•
The presence of any of these findings and/or the absence of fever should
suggest a diagnosis other than KD.

•
Measles, echovirus, adenovirus and EBV – These viral illnesses
may share many of the signs of mucocutaneous inflammation, but
they typically have less evidence of systemic inflammation and
generally lack the extremity changes seen in KD.

•
Toxin-mediated illnesses, especially group A streptococcal
infections (eg, scarlet fever and toxic shock syndrome) – These
usually lack the ocular and articular involvement typical of KD
•
Rocky Mountain spotted fever and leptospirosis – Headache and
gastrointestinal complaints typically are prominent features of these
infections.

•
Drug reactions such as SJS or serum sickness
•
Systemic juvenile idiopathic arthritis (JIA) – Children with this
condition generally lack the conjunctival and oral findings of KD.
Lymphadenopathy also is generalized, and it may be accompanied
by splenomegaly.

Delayed diagnosis
•
Treatment with intravenous immune globulin (IVIG) within the first
10 days of illness reduces the prevalence of coronary artery (CA)
aneurysms fivefold compared with children not treated with IVIG
.Thus, it is desirable to diagnose KD as soon as possible after the
onset of symptoms in order to initiate treatment and reduce the risk
of CA lesions

KD shock syndrome (KDSS)
•
defined as sustained systolic hypotension (decrease in blood
pressure greater than 20 percent from baseline) or clinical signs of
poor perfusion, is a potentially life-threatening complication .
•
It may be accompanied by multiple organ dysfunction syndrome
(MODS)
•
It is important to distinguish patients with KDSS from those with
other causes of severe hypoperfusion , Thrombocytosis
•
Younger age, Echocardiographic abnormalities

MACROPHAGE ACTIVATION
SYNDROME
•
activation and proliferation of macrophages and T cells. This may
lead to severe complications including disseminated intravascular
coagulopathy, cytopenias, and thrombosis.
•
It has been described rarely in children with KD who have
persistent and sustained fever after intravenous immune
globulin (IVIG) treatment
•
IVIG and systemic glucocorticoids are the first choice medications

CARDIAC COMPLICATIONS
•
major complication of KD is coronary artery abnormalities (CAAs),
which may result in myocardial ischemia, myocardial infarction, and
sudden death. Other cardiovascular complications include
decreased myocardial function in the acute phase, valvular
regurgitation, pericardial effusion, and peripheral artery aneurysms
(PAAs) .Infants <6 months old with KD have the highest risk of
developing cardiac complications.

•
In the first weeks after KD onset, approximately 25 percent of KD patients
overall and >50 percent of infants younger than age six months have
coronary aneurysms (ie, Z-scores ≥2.5) and approximately 1 percent
develop giant coronary aneurysms (ie, Z-scores ≥10 or absolute
dimension ≥8 mm)
•
CAAs typically increase over the first four to six weeks after illness onset,
and approximately half regress to normal lumen diameter over the
subsequent two years.
•
Patients treated with IVIG within the first 10 days of illness have an
approximately 75 percent decrease in the risk of developing coronary
artery aneurysms and a more than 95 percent decrease in mortality.

Factors associated with increased risk
of CAAs
•
Late diagnosis and delayed treatment with IVIG
•
●Age younger than one year or older than nine years
•
●Male sex
•
●Fever ≥14 days
•
●Serum sodium concentration <135 mEq/L
•
●Hematocrit <35 percent
•
●White blood cell count >12,000/mm3

NONCORONARY VASCULAR
INVOLVEMENT
•
Medium-sized muscular arteries (eg, axillary, iliac, brachial, and
mesenteric) are the most commonly involved, while small arteries,
veins, and visceral vessels are relatively spared.

Intravenous immune globulin
•
If IVIG started within 10 days of fever onset reduces the risk of CA
aneurysms from approximately 25 to <5 percent.
•
The mechanism of the beneficial effect of IVIG remains unknown.
IVIG appears to have a generalized anti-inflammatory effect.

Administration
•
IVIG is most effective when administered in a single infusion given
over 8 to 12 hours. This is illustrated by the following studies:
•
●A randomized trial of 549 patients with KD demonstrated that a
single dose of IVIG (2 g/kg), compared with a four-day treatment
regimen (400 mg/kg for four consecutive days), led to a more rapid
resolution of fever, normalization of laboratory evidence of acute
inflammation, and lower risk of CA abnormalities

Adverse effects
•
Flu-Like Symptoms
•
Dermatological Adverse Effects, urticaria, spot papules, eczema
•
Arrhythmia and Hypotension
•
hemolytic anemia ,occur within 5 to 10 days of infusion, due to
isoagglutinins in the IVIG products
•
transmission of bloodborne pathogens.
•
aseptic meningitis

Aspirin
•
unclear if the addition of aspirin provides greater anti-inflammatory
effects than does IVIG alone.
•
does not affect aneurysm formation

•
The dose of aspirin used to achieve an anti-inflammatory effect
during the acute phase of illness is relatively high, with a
recommended range of 30 to 100 mg/kg per day in four divided
doses
•
30 to 50 mg/kg per day (maximum 4 g/day), the lower end of the
dose range recommended by the AHA and AAP guidelines

•
Once fever has been absent for 48 hours, patients are generally
switched to a low dose of aspirin, 3 to 5 mg/kg per day, for its
antiplatelet effect. This low-dose aspirin regimen is continued until
laboratory markers of ongoing inflammation (eg, platelet count and
ESR) return to normal, unless CA abnormalities are detected by
echocardiography.
•
CRP usually normalizes within one to two weeks of IVIG treatment;
however, normalization of ESR typically takes an additional one to
two months.

Additional therapy for patients at high
risk for IVIG resistance Kobayashi score

Glucocorticoids
•
for KD in Japanese patients with a Kobayashi score ≥5
•
non-Japanese children who meet one or more of the high-risk
criteria for IVIG resistance.
•
Cyclosporine

PROGNOSIS
•
Mortality due to KD is rare among children treated with
intravenous (IVIG). Long-term morbidity is primarily related to the
degree of coronary artery (CA) involvement. Recurrence of KD is
uncommon.
•
The reported mortality rate is (0.1 to 0.3 percent) since the advent
of IVIG therapy .The rare fatal outcomes from severe cardiac
involvement in KD are generally the result of either myocardial
infarction or arrhythmias, although aneurysm rupture can also
occur. Mistaken or late diagnosis, or complete lack of IVIG
treatment, is associated with potentially fatal outcomes

Long-term morbidity
•
depends upon the severity of CA involvement.
•
Children without cardiovascular abnormalities detected in the acute
and subacute phase (up to eight weeks after onset of disease)
appear to be clinically asymptomatic 10 to 21 years later
•
However, the long-term effect on cardiovascular health is unknown

•
CA dilatation <8 mm generally regresses over time
•
Patients with giant aneurysms (maximum diameter ≥8 mm) are at
the greatest risk for myocardial infarction resulting from CA
occlusion.

Recurrence
•
2 percent of patients
•
The highest incidence was in children less than three years of age
who had cardiac sequelae during the first episode. Recurrences
most commonly occurred within the first 12 months after the initial
episode of KD.

FOLLOW-UP
•
Follow-up after discharge includes monitoring for recurrence of
fever and repeat echocardiograms to assess for cardiac
involvement. Repeat echocardiograms are usually obtained at one to
two weeks and again four to six weeks after discharge.
•
Live-virus vaccines are postponed due to decreased
immunogenicity in children who have received (IVIG) treatment.
•
should be postponed for at least 11 months in children who have
been treated with IVIG.

•
influenza immunization, recommended in all children over six
months of age, is particularly important in those who require long-
term high-dose aspirin therapy because of the possible increased
risk of Reye syndrome.

References
•
https://www.uptodate.com/contents/kawasaki-disease-initial-treatment-and-
prognosis?search=Kawasaki%20disease&source=search_result&selectedTitle=2~15
0&usage_type=default&display_rank=2#H401495920
•
https://www.uptodate.com/contents/kawasaki-disease-epidemiology-and-
etiology?search=Kawasaki%20disease&source=search_result&selectedTitle=4~150
&usage_type=default&display_rank=4
•
https://www.uptodate.com/contents/kawasaki-disease-
complications?search=Kawasaki%20disease&source=search_result&selectedTitle=5
~150&usage_type=default&display_rank=5

•
https://www.uptodate.com/contents/kawasaki-disease-clinical-features-and-
diagnosis?search=Kawasaki%20disease&source=search_result&selectedTitle=1~15
0&usage_type=default&display_rank=1
•
https://www.uptodate.com/contents/incomplete-atypical-kawasaki-
disease?search=Kawasaki%20disease&source=search_result&selectedTitle=3~150
•
https://www.uptodate.com/contents/refractory-kawasaki-
disease?search=Kawasaki%20disease&source=search_result&selectedTitle=7~150

•
https://www.slideshare.net/SidKaithakkoden/kawasaki-disease-45143553
•
https://pubmed.ncbi.nlm.nih.gov/21343843/

Kawasaki disease Dr. Abdelqader wishah

Kawasaki disease Dr. Abdelqader wishah

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