Kawasaki Disease: Pathology
Kawasaki Disease: Pathology
Kawasaki Disease: Pathology
DISEASE
• Formerly known as mucocutaneous PATHOLOGY
lymph node syndrome and infantile • Predominantly affects medium-sized
polyarteritis nodosa arteries
• Highest incidence in Asian children • Coronary arteries: most commonly
• Vasculitis with a predilection for involved, others: popliteal and brachial
coronary arteries • 3 phase process to arteriopathy of KD:
• Untreated children: develop coronary o 1st phase – neutrophilic
artery abnormalities including necrotizing arteritis occurring in
aneurysm; If treated with IVIG <5% will the 1st 2 weeks of illness that
develop CAA begins in endothelium and
moves through the coronary
ETIOLOGY wall
• Unknown, but certain epidemiologic - Saccular aneurysms
and clinical features support an may form
infectious origin o 2nd phase – subacute/chronic
vasculitis driven by
• Features: lymphocytes, plasma cells, and
o Young age group eosinophils which may last to
o Wave-like geographic spread of weeks or years
illness - Fusiform
o Self-limited nature of acute aneurysms
febrile illness o 3rd phase – smooth muscle cell
o Clinical features of rash, fever, myofibroblast will develop
enanthem, conjunctival which can cause progressive
injection, cervical stenosis
lymphadenopathy - Thrombi may form
o Infrequent if <3 mo d/t in the lumen and
maternal Ab obstruct blood flow
o No single infectious etiologic
agent has been identified MANIFESTATIONS
o Genetics: ITPKC gene- increased • high fever (>38.3°C), unremitting and
susceptibility and more severe unresponsive to antibiotics lasting
disease atleast 5 days. If no treatment, fever
may last up to 4 weeks
EPIDEMIOLOGY • In addition to fever , 5 criteria
• M > F; mean age 3 yo o Bilateral non-exudative
• Kobayashi score: high sensitivity and conjunctival injection with
specificity on Japanese population only limbal sparing
• Predictors of poor outcome: o Erythema of oral and
o Young age pharyngeal mucosa with
o Male strawberry tongue and red
o Persistent fever cracked lips
o Poor response to IVIG o Non-suppurative cervical
o Lab abn: WBC, ¯plt, ¯Na. lymphadenopathy, usually
¯albumin,CRP, transaminitis unilateral with node size >1.5
cm
KAWASAKI DISEASE
o Edema and erythema of the v Myocarditis occurs in
hands and feet most and manifest as
o Rash of various forms tachycardia
(maculopapular, erythema disproportionate to
multiforme or scarlantiniform) fever, along with
diminished left
• Symptoms other than criteria: ventricular systolic
o GI symptoms: vomiting, function
diarrhea or abd pain v KD shock syndrome –
o Respi symptoms: rhinorrhea or KD patients with
cough cardiogenic shock
o Irritability which may be a v Pericarditis with small
consequence of aseptic pericardial effusion
meningitis, mild hepatitis, v Mitral regurgitation
hydrops of the gallbladder, v Giant coronary
urethritis, and meatitis with aneurysms (>8mm
sterile pyuria and arthritis internal diameter) –
o Localized pulsating mass – greatest risk for
dilatation of popliteal, axillary, rupture, stenosis, and
iliac or other arteries MI
o Cardiac involvement – most Node-first KD – 2-4 yo with fever and
important manifestation lymphadenopathy presentation only
KAWASAKI DISEASE
KAWASAKI DISEASE
3 phase process:
Acute febrile phase Subacute phase Convalescent phase
Fever and other acute signs that Desquamation, thrombocytosis All clinical signs have
lasts up to 2 wks and disappeared and continues until
CAA develop in up to 25% of ESR returns to normal 6-8 wks
perineal desquamation untreated patients after onset
arthritis and periungual
desquamation of fingers and
toes which may progress the
entire hand and foot
Laboratory and Radiology Findings
• 2D echo - most useful test to monitor CAA. Aneurysms may be small (<5mm internal diameter),
or giant (>8 mm internal diameter)
- Should be done at diagnosis and again after 2-3 wks of illness. If normal, a
repeat should be done 6-8 wks after
- If abn 2D echo or with recurrent fever – more frequent 2d echo
- 1 yr later – 2d echo and lipid profile then cardio follow up every 5 yrs
• WBC with predominance of neutrophils and immature forms
• Normochromic anemia
• Plt – normal in 1st wk then in 2-3 wks
• ESR and CRP
• Sterile pyuria, mild elevations of hepatic transaminases, hyperbilirubinemia, CSF pleocytosis
DIAGNOSIS
• Based on presence of clinical signs
Classic KD Atypical or Incomplete
Presence of fever for atleast 4 days and Fever but fewer than 4 of the 5
atleast 4 of 5 of other principal characteristics
Infants – highest likelihood of CAA
Do labs and 2d echo to assist in dx
KAWASAKI DISEASE
KAWASAKI DISEASE
KAWASAKI DISEASE
TREATMENT • Persistent or recrudescent fever
A.IVIG 36h after completion of the
• 2g/kg of IVIG and high dose aspirin 80- intial IVIG infusion
100 mg/kg/day Q6 within 10 days of • Have increased risk for CAA
disease onset • Give another dose of IVIG 2g/kg
• IVIG results in defervescence and
resolution of clinical signs of illness in COMPLICATIONS
85% of patients • Small solitary aneurysm – continue
• Defer MMRV vaccine until 11 mos after Aspirin indefinitely
IVIG administration. Non-live vaccines • Larger or numerous aneurysm – require
do not need to be deferred addition of other antiplatelet agents or
anticoagulation
B.Aspirin • Long term: periodic echo with stress
• decreased from anti-inflammatory dose testing and possible angiography if large
to anti-thrombotic dose (3-5 mg/kg/day aneurysm are present
as a single dose) after the patient has • Coronary stenosis: catheter
been afebrile for 48 hrs, although some intervention with percutaneous
prescribe until 14th day of illness transluminal coronary rotational
• continued for 6-8 wks after illness onset ablation, directional coronary
and dc in patients who have had normal atherectomy, and stent implantation
2d echo
• If with CAA, Aspirin is continued and PROGNOSIS
may require anticoagulation, depending • Depends on the severity of coronary
on the degree of coronary dilation disease
• If long term: give annual Influenza • Acute KD recurs in 1-3% of cases
vaccination to reduce Reye syndrome • 50% of coronary aneurysms regress to
normal lumen diameter by 1-2 yrs after
C.Corticosteroids the illness, with smaller aneurysms
Single pulse dose of IV methylprednisone more likely to regress
2mg/kg plus IVIG • Giant aneurysms are less likely to
regress and may lead to thrombosis and
IVIG-resistant KD stenosis. CABG( arterial grafts) may be
• Occurs in 15% of patients required if myocardial perfusion is
impaired