1. Kawasaki Disease
Kawasaki disease (KD), formerly known as mucocutaneous
lymph node syndrome and infantile polyarteritis nodosa, is
an acute febrile vasculitis of childhood first described by Dr.
Tomisaku Kawasaki in Japan in 1967. The disorder occurs
worldwide, with Asians at highest risk. Approximately 20% of
untreated patients develop coronary artery abnormalities
including aneurysms, with the potential for severely affected
patients to develop coronary artery thrombosis or stenosis,
myocardial infarction, aneurysm rupture, and sudden death.
Kawasaki disease is the leading cause of acquired heart
disease in children in the United States and Japan.
ETIOLOGY.
The cause of the illness remains unknown, but epidemiologic
and clinical features strongly support an infectious origin.
These features include the young age group affected,
epidemics with wavelike geographic spread of illness, the
self-limited nature of the acute febrile illness, and the
combination of clinical features of fever, rash, enanthem,
conjunctival injection, and cervical lymphadenopathy.
Nonetheless, it is unusual to have multiple cases present at
the same time from a family or day care center. One
hypothesis is that a ubiquitous childhood infectious agent
causes Kawasaki disease and that symptomatic illness
occurs only in genetically predisposed persons. The
infrequent occurrence of the illness in infants <3 mo may be
the result of maternal antibody, and the virtual absence of
cases in adults may be due to widespread immunity. A KD-
associated antigen may be present in cytoplasmic inclusion
bodies within ciliated bronchial epithelial cells of acute fatal
cases. These inclusions appear consistent with viral protein
aggregates, and support the hypothesis of a respiratory

2. portal of entry of the KD agent. Kawasaki disease has
recurred in families when previously affected parents have
children who develop the disease. Genetic variation of
CCR5, which encodes a high-affinity receptor for the
chemokines CCL3 and CCL3L1, suggests an influential role
of gene-gene interactions for susceptibility to Kawasaki
disease.
EPIDEMIOLOGY.
An estimated 3,000 cases are diagnosed annually in the
United States. The incidence of Kawasaki disease in Asian
children is substantially higher than in other racial groups,
but the illness occurs worldwide in all ethnic groups. Most
cases in the United States occur in white and black children,
reflecting the racial composition of the population. In Japan,
almost 200,000 cases have been reported since the 1960s.
Kawasaki disease is not a new illness; infantile periarteritis
nodosa was an autopsy diagnosis before the 1960s that
appears in retrospect to have represented fatal Kawasaki
disease. The disorder bears marked clinical similarities to
measles and may have been particularly difficult to identify
clinically before widespread immunization with measles
vaccine. The illness occurs predominantly in young children;
80% of patients are <5 yr, and, only occasionally, are
teenagers or, more rarely, adults affected. More often,
affected adolescents or adults may actually meet the criteria
for toxic shock syndrome, which has some similar features to
Kawasaki disease.
PATHOGENESIS.
Kawasaki disease causes severe vasculitis of all blood
vessels but predominantly affects the medium-sized arteries,
with a striking predilection for the coronary arteries.
Pathologic examination of fatal cases in the acute or

3. subacute stages reveals edema of endothelial and smooth
muscle cells with intense inflammatory infiltration of the
vascular wall, initially by polymorphonuclear cells but rapidly
followed by macrophages, lymphocytes (primarily CD8 T
cells), and plasma cells. IgA plasma cells are particularly
prominent in the inflammatory infiltrate. In the most severely
affected vessels, inflammation involves all three layers of the
vascular wall with destruction of the internal elastic lamina.
The vessel loses its structural integrity and weakens,
resulting in dilatation, or saccular or fusiform aneurysm
formation. Thrombi may form in the lumen and obstruct
blood flow. In the healing phase, the vascular wall can
become progressively fibrotic with marked intimal
proliferation, which may lead to stenotic occlusion of the
vessel over time.
In acute Kawasaki disease, an inflammatory infiltrate,
including IgA plasma cells, is present in certain nonvascular
tissues including myocardium, upper respiratory tract,
pancreas, kidney, and biliary tract, suggesting that the
infectious agent may cause a host immune response in a
variety of tissues. No significant sequelae appear to occur in
any of these nonvascular tissues after resolution of the acute
illness.
Elevated serum levels of all immunoglobulins develop during
the subacute phase of illness, suggesting that a vigorous
antibody response occurs. It is unclear whether the etiologic
agent, the host immune response, or both are the major
causal factors leading to coronary artery disease.
CLINICAL MANIFESTATIONS.
Fever is characteristically high (104°F or higher), remittent,
and unresponsive to antibiotics. The duration of fever without
treatment is generally 1–2 wk, but it may persist for 3–4 wk.

4. Prolonged fever is prognostic for the development of
coronary artery disease. In addition to fever, the five
characteristic features of Kawasaki disease are: bilateral
bulbar conjunctival injection, usually without exudate;
erythema of the oral and pharyngeal mucosa with strawberry
tongue and dry, cracked lips, and without ulceration; edema
and erythema of the hands and feet; rash of various forms
(maculopapular, erythema multiforme, or scarlatiniform) with
accentuation in the groin area; and nonsuppurative cervical
lymphadenopathy, usually unilateral, with node size of ≥1.5
cm ( Table 165-1 , Figs. 165-1, 165-2, 165-3, and 165-4 [1]
[2] [3] [4]). Perineal desquamation is common in the acute
phase. Periungual desquamation of the fingers and toes
begins 1–3 wk after the onset of illness and may progress to
involve the entire hand and foot ( Fig. 165-5 ).
TABLE 165-1 -- Clinical and Laboratory Features of
Kawasaki Disease
EPIDEMIOLOGIC CASE DEFINITION (CLASSIC CLINICAL
CRITERIA)[*]
Fever persisting at least 5 days[†]
Presence of at least 4 principal features:
Changes in extremities
Acute: Erythema of palms, soles; edema of hands, feet
Subacute: Periungual peeling of fingers, toes in weeks 2
and 3
Polymorphous exanthem
Bilateral bulbar conjunctival injection without exudate
Changes in lips and oral cavity: Erythema, lips cracking,
strawberry tongue, diffuse injection of oral and pharyngeal

5. mucosae
Cervical lymphadenopathy (>1.5 cm diameter), usually
unilateral
Exclusion of other diseases with similar findings[‡]
OTHER CLINICAL AND LABORATORY FINDINGS
Cardiovascular findings
Congestive heart failure, myocarditis, pericarditis, valvular
regurgitation
Coronary artery abnormalities
Aneurysms of medium-size noncoronary arteries
Raynaud phenomenon
Peripheral gangrene
Musculoskeletal system
Arthritis, arthralgia
Gastrointestinal tract
Diarrhea, vomiting, abdominal pain
Hepatic dysfunction
Hydrops of gallbladder
Central nervous system
Extreme irritability
Aseptic meningitis
Sensorineural hearing loss
Genitourinary system
Urethritis/meatitis
Other findings

6. Erythema, induration at BCG inoculation site
Anterior uveitis (mild)
Desquamating rash in groin
LABORATORY FINDINGS IN ACUTE KAWASAKI DISEASE
Leukocytosis with neutrophilia and immature forms
Elevated erythrocyte sedimentation rate (ESR)
Elevated C-reactive protein (CRP)
Anemia
Abnormal plasma lipids
Hypoalbuminemia
Hyponatremia
Thrombocytosis after week 1[§]
Sterile pyuria
Elevated serum transaminases
Elevated serum gamma glutamyl transpeptidase
Pleocytosis of cerebrospinal fluid
From Newburger JW, Takahashi M, Gerber MA, et al:
Diagnosis, treatment, and long-term management of
Kawasaki disease. Pediatrics 2004;114:1708–1733.
*
Patients with fever at least 5 days and <4 principal criteria
can be diagnosed with Kawasaki disease when coronary
artery abnormalities are detected by 2DE or angiography.
†
In the presence of e4 principal criteria, Kawasaki disease
diagnosis can be made on day 4 of illness. Experienced
clinicians who have treated many Kawasaki disease
patients may establish diagnosis before day 4.
‡
See differential diagnosis.

7. §
Some infants present with thrombocytopenia and
disseminated intravascular coagulation.
Figure 165-1 Clinical symptoms and signs of Kawasaki
disease. A summary of the clinical features from 110 cases of
Kawasaki disease seen in Kaohsiung, Taiwan. LAP indicates
lymphadenopathy in head and neck area; BCG, reactivation of
BCG site; CAD, coronary artery dilatation defined by an internal
diameter of >3 mm. (From Wang CL, Wu YT, Liu CA, et al:
Kawasaki disease: Infection, immunity and genetics. Pediatr
Infect Dis J 2005;24:998–1004.)

8. Figure 165-2 Strawberry tongue in mucocutaneous lymph
node syndrome (Kawasaki disease). (Courtesy of Tomisaku
Kawasaki, MD.) (From Hurwitz S: Clinical Pediatric
Dermatology, 2nd ed. Philadelphia, WB Saunders, 1993, p
545.)

9. Figure 165-3 Congestion of bulbar conjunctiva in a patient with
mucocutaneous lymph node syndrome (Kawasaki disease).
(Courtesy of Tomisaku Kawasaki, MD.) (From Hurwitz S:
Clinical Pediatric Dermatology, 2nd ed. Philadelphia, WB
Saunders, 1993, p 545.)

10. Figure 165-4 Indurative edema of the hands in
mucocutaneous lymph node syndrome (Kawasaki disease).
(Courtesy of Tomisaku Kawasaki, MD.) (From Hurwitz S:
Clinical Pediatric Dermatology, 2nd ed. Philadelphia, WB
Saunders, 1993, p 545.)

11. Figure 165-5 Desquamation of the fingers in a patient with
mucocutaneous lymph node syndrome (Kawasaki disease).
(Courtesy of Tomisaku Kawasaki, MD.) (From Hurwitz S:
Clinical Pediatric Dermatology, 2nd ed. Philadelphia, WB
Saunders, 1993, p 549.)
Other features include extreme irritability that is especially
prominent in infants, aseptic meningitis, diarrhea, mild
hepatitis, hydrops of the gallbladder, urethritis and meatitis
with sterile pyuria, otitis media, and arthritis. Arthritis may
occur early in the illness or may develop in the 2nd–3rd
week, generally affecting hands, knees, ankles, or hips. It is
self-limited but may persist for several weeks.

12. Cardiac involvement is the most important manifestation of
Kawasaki disease. Myocarditis, manifested as tachycardia
out of proportion to fever occurs in at least 50% of patients;
decreased ventricular function occurs in a smaller number of
patients. Pericarditis with a small pericardial effusion is
common during the acute illness. Coronary artery
aneurysms develop in up to 25% of untreated patients in the
2nd–3rd wk of illness and are best detected by two-
dimensional echocardiography. Giant coronary artery
aneurysms (≥8 mm internal diameter) pose the greatest risk
for rupture, thrombosis or stenosis, and myocardial infarction
( Fig. 165-6 ). Significant valvular regurgitation and systemic
artery aneurysms may occur but are uncommon. Axillary,
popliteal, or other arteries may also be involved and manifest
as a localized pulsating mass.

13. Figure 165-6 Coronary angiogram demonstrating giant
aneurysm of the LAD with obstruction and giant aneurysm of
the RCA with area of severe narrowing in 6 yr old boy. (From
Newburger JW, Takahashi M, Gerber MA, et al: Diagnosis,
treatment, and long-term management of Kawasaki disease.
Pediatrics 2004; 114:1708–1733.)
Kawasaki disease is generally divided into three clinical
phases. The acute febrile phase, which usually lasts 1–2 wk,
is characterized by fever and the other acute signs of illness.
The dominant cardiac manifestation is myocarditis. In
addition, a macrophage activation syndrome may rarely be
evident (see Chapter 154 ). The subacute phase begins
when fever and other acute signs have abated, but irritability,
anorexia, and conjunctival injection may persist. The
subacute phase is associated with desquamation,
thrombocytosis, the development of coronary aneurysms,
and the highest risk of sudden death in those who have
developed aneurysms. This phase generally lasts until about
the 4th wk. The convalescent phase begins when all clinical
signs of illness have disappeared and continues until the
erythrocyte sedimentation rate (ESR) and C-reactive protein
(CRP) return to normal, ≈6–8 wk after the onset of illness.
Certain clinical and laboratory findings may predict a more
severe outcome. These include male gender, age <1 yr,
prolonged fever, recrudescence of fever after an afebrile
period, and the following laboratory values at presentation:
low hemoglobin or platelet levels, high neutrophil and band
counts, hyponatremia, and low albumin and age-adjusted
serum IgG levels. Scoring systems based on these factors,

14. however, have not proven sufficiently sensitive for selective
treatment of patients based on risk.
DIAGNOSIS.
The diagnosis of Kawasaki disease is based on the
presence of characteristic clinical signs. For classic
Kawasaki disease, the diagnostic criteria require the
presence of fever for at least 5 days and at least four of five
of the other characteristic clinical features of illness (see
Table 165-1 ). In atypical or incomplete Kawasaki disease,
the patient has persistent fever but with fewer than four other
features of the illness. Accurate identification of incomplete
cases is a major clinical challenge ( Fig. 165-7 ). Incomplete
cases are most frequent in infants, who, unfortunately, also
have the highest likelihood of developing coronary artery
disease.

15. Figure 165-7 Evaluation of suspected incomplete
Kawasaki disease (KD). (1) In the absence of gold
standard for diagnosis, this algorithm cannot be evidence
based but rather represents the informed opinion of the
expert committee. Consultation with an expert should be
sought anytime assistance is needed. (2) Infants ≤6
months old on day ≥7 of fever without other explanation
should undergo laboratory testing and, if evidence of

16. systemic inflammation is found, an echocardiogram, even
if the infants have no clinical criteria. (3) Patient
characteristics suggesting Kawasaki disease are listed in
Table 165-1 . Characteristics suggesting disease other
than Kawasaki disease include exudative conjunctivitis,
exudative pharyngitis, discrete intraoral lesions, bullous or
vesicular rash, or generalized adenopathy. Consider
alternative diagnoses. (4) Supplemental laboratory criteria
include albumin ≤3.0 g/dL, anemia for age, elevation of
alanine aminotransferase, platelets after 7 days ≥450,000/
mm3, white blood cell count ≥15,000/mm3, and urine ≥10
white blood cells/high-power field. (5) Can treat before
performing echocardiogram. (6) Echocardiogram is
considered positive for purposes of this algorithm if any of
3 conditions are met: z score of LAD or RCA ≥2.5,
coronary arteries meet Japanese Ministry of Health criteria
for aneurysms, or ≥3 other suggestive features exist,
including perivascular brightness, lack of tapering,
decreased left ventricle (LV) function, mitral regurgitation,
pericardial effusion, or z scores in LAD or RCA of 2–2.5.
(7) If the echocardiogram is positive, treatment should be
given to children within 10 days of fever onset and to those
beyond day 10 with clinical and laboratory signs (CRP,
ESR) of ongoing inflammation. (8) Typical peeling begins
under nail bed of fingers and then toes. (From Newburger
JW, Takahashi M, Gerber MA, et al: Diagnosis, treatment,
and long-term management of Kawasaki disease.
Pediatrics 2004;114:1708–1733.)
Recognition depends on a high index of suspicion and
knowledge of the characteristic clinical features.
Unfortunately, if the diagnosis is not established and

17. treatment is not instituted, rare patients may suffer sudden
death secondary to myocardial infarction or coronary
aneurysm rupture, or may develop serious asymptomatic
coronary disease that is unrecognized until symptoms of
myocardial ischemia develop later in life.
DIFFERENTIAL DIAGNOSIS.
The differential diagnosis of Kawasaki disease includes
scarlet fever, toxic shock syndrome, measles, adenovirus
infection, drug hypersensitivity reactions including Stevens-
Johnson syndrome, juvenile rheumatoid arthritis, and, more
rarely, Rocky Mountain spotted fever and leptospirosis.
Some features of measles that distinguish it from Kawasaki
disease include exudative conjunctivitis, Koplik spots, rash
that begins on the face and behind the ears, leukopenia, and
a normal ESR and/or CRP. Adenovirus infection is
associated with exudative pharyngitis and exudative
conjunctivitis, unlike Kawasaki disease. Some features of
drug reactions, such as the presence of periorbital edema,
oral ulcerations, and a normal or minimally elevaled ESR
may help to distinguish these reactions from Kawasaki
disease. Toxic shock syndrome is distinguished by the
presence of hypotension, renal and hepatic dysfunction,
elevated creatine phosphokinase level, and focal
Staphylococcus aureus infection. A common clinical problem
is the differentiation of scarlet fever from Kawasaki disease
in a child who is a group A streptococcal carrier. Because
patients with scarlet fever have a rapid clinical response to
appropriate antibiotic therapy, such treatment for 24–48 hr
with clinical reassessment at that time generally clarifies the
diagnosis. The presence of lymphadenopathy,
hepatosplenomegaly, and an evanescent, salmon-colored
rash suggests a diagnosis of juvenile rheumatoid arthritis.
Accurate diagnosis of incomplete Kawasaki disease remains

18. a significant challenge for clinicians. Unusual cases should
be referred to a center with experience in the diagnosis of
Kawasaki disease.
LABORATORY FINDINGS.
There is no diagnostic test for Kawasaki disease, but certain
laboratory findings are characteristic. The leukocyte count is
normal to elevated with a predominance of neutrophils and
immature forms. Elevated ESR, CRP, and other acute phase
reactants are almost universally present in the acute phase
of illness and may persist for 4–6 wk. Normocytic,
normochromic anemia is common. The platelet count is
generally normal in the 1st week of illness and rapidly
increases by the 2nd–3rd wk of illness, sometimes
exceeding 1,000,000/mm3. Tests for antinuclear antibody
and rheumatoid factor are negative. Sterile pyuria, mild
elevations of the hepatic transaminases, and cerebrospinal
fluid pleocytosis may be present.
Two-dimensional echocardiography, which should be
performed by a pediatric cardiologist, is the most useful test
to monitor potential development of coronary artery
abnormalities. Echocardiography should be performed at
diagnosis and again after 2–3 wk of illness. If both are
normal, a repeat study should be performed 6–8 wk after
onset of illness. If coronary abnormalities are not detected by
6–8 wk after onset of illness, and after the ESR has
normalized, additional follow-up studies are optional. Some
centers routinely perform echocardiography again 6 or 12
mo after onset of illness. Kawasaki disease is an acute
vasculitis; there is no convincing evidence of long-term
cardiovascular sequelae in children who do not develop
coronary abnormalities within 2 mo after the onset of illness.

19. For patients who develop coronary artery abnormalities,
more frequent echocardiographic studies and, potentially,
angiography may be indicated. Treatment of such patients
should be determined in consultation with a pediatric
cardiologist.
TREATMENT.
Patients with acute Kawasaki disease should be treated with
intravenous immunoglobulin (IVIG) and high-dose aspirin as
soon as possible after diagnosis and, ideally, within 10 days
of disease onset ( Table 165-2 ). The mechanism of action of
IVIG in Kawasaki disease is unknown, but treatment should
result in rapid defervescence and resolution of clinical signs
of illness in 85–90% of patients. With therapy, the CRP
normalizes much more quickly than the ESR, which will often
increase immediately after IVIG therapy. IVIG reduces the
prevalence of coronary disease from 20–25% in children
treated with aspirin alone to 2–4% in those treated with IVIG
and aspirin within the 1st 10 days of illness. Consideration
should even be given to treatment of patients diagnosed
after the 10th illness day if fever has persisted, because the
anti-inflammatory effect may be helpful, although the effect
of such therapy on the risk of developing coronary
aneurysms is unknown. The dose of aspirin is decreased
from anti-inflammatory to antithrombotic doses (3–5
mg/kg/day as a single dose) on the 14th illness day or after
the patient has been afebrile for at least 3–4 days. Aspirin is
continued for its antithrombotic effect until 6–8 wk after
onset, when the ESR has normalized in patients that have
not developed abnormalities detected by echocardiography.
TABLE 165-2 -- Treatment of Kawasaki Disease
ACUTE STAGE

20. Intravenous immunoglobulin 2 g/kg over 10–12 hr with aspirin
80–100 mg/kg/day divided every 6 hr orally until 14th illness
day
CONVALESCENT STAGE
Aspirin 3–5 mg/kg once daily orally until 6–8 wk after illness
onset
LONG-TERM THERAPY FOR THOSE WITH CORONARY
ABNORMALITIES
Aspirin 3–5 mg/kg once daily orally ± clopidogrel 1 mg/kg/day
(max 75 mg/day) (most experts add warfarin for those
patients at particularly high risk of thrombosis)
ACUTE CORONARY THROMBOSIS
Prompt fibrinolytic therapy with tissue plasminogen activator,
streptokinase, or urokinase under supervision of a pediatric
cardiologist
Occasional patients have refractory Kawasaki disease that
does not respond to an initial IVIG infusion, or exhibits only a
partial or transient response. Strong consideration should be
given to retreatment of these patients with an additional
infusion of IVIG (2 g/kg). Optimal therapy for patients who do
not respond to two infusions of IVIG is uncertain. If there is a
poor response to the second dose of IVIG, some patients
have responded to IV methylprednisolone at a dose of 30
mg/kg/day for 3 days. The value of infliximab (which binds
TNF-α), other immunomodulators (cyclophosphamide,
methotrexate), and plasmapheresis in Kawasaki disease is
yet to be determined.

21. Acute thrombosis may occasionally occur in an aneurysmal
or stenotic coronary artery. Thrombolytic therapy may be
lifesaving in this circumstance ( Table 165-3 ). Abciximab, a
glycoprotein IIb/IIIa inhibitor, has been used in some patients
with Kawasaki disease who develop giant coronary
aneurysms with possible thrombosis. Few data regarding
efficacy are available, but the drug may reduce thrombotic
complications.
TABLE 165-3 -- Antiplatelet, Anticoagulant, and
Thrombolytic Medications
MEDICATION ROUTE DOSAGE
Aspirin Oral Antiplatelet dose: 3–5 mg/kg
every day
Clopidogrel Oral 1 mg/kg per day[*] to max
(adult dose) of 75 mg/day
Dipyridamole Oral 2–6 mg/kg per day in 3
divided doses[†]
Unfractionated Intravenous Load: 50 U/kg
heparin
Infusion: 20 U/kg/h
Adjust dosage to achieve
desired therapeutic level,
usually plasma heparin level
= 0.35–0.7 in antifactor Xa
activity or aPTT 60–85 s
Low molecular Subcutaneous Infants <12 months
weight heparin
Treatment: 3 mg/kg per day,
divided 12 h

22. MEDICATION ROUTE DOSAGE
Prophylaxis: 1.5 mg/kg per
day, divided every 12 h
Children/adolescents
Treatment: 2 mg/kg per day,
divided every 12 h
Prophylaxis: 1 mg/kg per
day, divided every 12 h
Adjust dose to achieve
desired therapeutic level,
usually antifactor Xa = 0.5–
1.0 U/mL
Abciximab Intravenous Bolus: 0.25 mg/kg
Infusion: 0.125 μg/kg per min
for 12 h
Streptokinase Intravenous Bolus: 1000–4000 U/kg over
30 min
Infusion: 1000–1500 U/kg per
h
tPA Intravenous Bolus: 1.25 mg/kg
Infusion: 0.1–0.5 mg/kg per h
for 6 h, then reassess
Urokinase Intravenous Bolus: 4400 U/kg over 10 min
Infusion: 4400 U/kg per h
Warfarin Oral 0.1 mg/kg per day, given
every day (0.05–0.34 mg/kg
per day; adjust dose to
achieve desired INR, usually
2.0–2.5)

23. From Newburger JW, Takahashi M, Gerber MA, et al:
Diagnosis, treatment, and long-term management of
Kawasaki disease. Pediatrics 2004;114:1708–1733.
*
No published studies in children.
†
Clopidogrel is preferred to dipyridamole, based on adult
studies.
Patients with a small solitary aneurysm should continue
aspirin indefinitely. Patients with larger or numerous
aneurysms may require the addition of clopidogrel, warfarin,
or low molecular weight heparin therapy; such decisions
should be made in consultation with a pediatric cardiologist.
Long-term follow-up of patients with coronary artery
aneurysms should include periodic echocardiography with
stress testing, and possibly angiography for those with larger
aneurysms. Catheter intervention with percutaneous
transluminal coronary rotational ablation, directional
coronary atherectomy, and stent implantation have all been
used for the management of coronary stenosis caused by
Kawasaki disease, with some patients requiring coronary
artery bypass grafting.
Patients receiving long-term aspirin therapy are candidates
for annual influenza vaccination to reduce the risk of Reye
syndrome. Varicella vaccination should be strongly
considered, since the risk of Reye syndrome in children who
take salicylates and who receive varicella vaccine is likely to
be lower than with wild-type varicella. Patients treated with 2
g/kg IVIG should have measles-mumps-rubella and varicella
vaccinations delayed for 11 mo because the specific antiviral
antibody in IVIG may interfere with the immune response to
live-virus vaccines. Other vaccinations do not need to be
delayed.

24. COMPLICATIONS AND PROGNOSIS.
Recovery is complete and without apparent long-term effects
for patients who do not develop coronary disease. Recurrent
acute illness occurs in only 1–3% of cases. The prognosis
for patients with coronary abnormalities depends on the
severity of coronary disease. In Japan, fatality rates are very
low, about 0.01%. Overall, 50% of coronary artery
aneurysms resolve as assessed by echocardiogram 1–2 yr
after the illness. Intravascular ultrasonography has
demonstrated, however, that resolved aneurysms are
associated with marked myointimal thickening and abnormal
functional behavior of the vessel wall. Giant aneurysms are
unlikely to resolve and are most likely to lead to thrombosis
or stenosis. Coronary artery bypass grafting may be required
if myocardial perfusion is significantly impaired, and it is best
accomplished with the use of arterial grafts, which grow with
the child and are more likely to remain patent long-term than
are venous grafts. Heart transplantation has been required in
rare cases in which revascularization is not feasible because
of distal coronary stenoses, distal aneurysms, or severe
myocardial dysfunction. Whether the presence of coronary
artery abnormalities resulting from Kawasaki disease
predisposes to the development of atherosclerotic heart
disease in young adulthood is unknown, although long-term
followup studies in Japan, to date, provide little if any
evidence of this.