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CLINICAL OVERVIEW
Kawasaki Disease
Elsevier Point of Care (ver detalles)
Actualizado July 20, 2021. Copyright Elsevier BV. All rights reserved.
Synopsis
Key Points Urgent Action

Kawasaki disease is an acute, self-limited, acquired Although the disease is
inflammatory disorder characterized by vasculitis of small to self-limited, treatment
medium vessels that can lead to coronary artery aneurysms, should start within 10 days
usually in children of fever onset to minimize
risk for coronary artery
Complete Kawasaki disease is a diagnosis based on findings
aneurysm formation
of fever for 5 or more days and at least 4 of 5 other key
historical or physical findings, as follows: Children with Kawasaki
disease can rarely present
Rash (polymorphous but not vesicular or bullous)
with significant signs of
Oropharyngeal mucous membrane changes myocarditis, shock, heart
failure, impaired
Conjunctival injection ventricular function, and
ischemic heart disease as a
Extremity changes result of inflammatory
vasculitic changes in and
Lymphadenopathy around the heart. These
children should be
Incomplete Kawasaki disease is a dangerous diagnostic promptly evaluated and
dilemma, as children present with prolonged fever and do not stabilized in a pediatric
meet all of the clinical criteria for complete Kawasaki disease ICU
diagnosis
Esquema
Serial physical examinations with supporting laboratory results (eg, increased levels of
inflammatory markers, such as C-reactive protein level and erythrocyte sedimentation rate)
lend weight toward a diagnosis of incomplete Kawasaki disease when differential diagnosis is
challenging because some criteria are not met at initial presentation (some of the 5 cardinal
signs may not occur until later)
All children in whom Kawasaki disease is suspected require ECG, echocardiogram, and urgent
consultation with pediatric cardiologist
Primary treatment is IV immunoglobulin and aspirin. For patients at high risk of coronary
artery aneurysms (ie, those with longer duration of fever and/or delay in treatment), the
addition of a corticosteroid regimen (along with specialist consultation) is suggested
Most children with Kawasaki disease who have minimal coronary involvement will return to
baseline state of health if successfully treated within 10 days of onset of fever
In patients with Kawasaki disease with aneurysms on echocardiogram, ongoing monitoring

and management depend on stratification of risk for development of myocardial infarction
and are tailored to the type and degree of coronary involvement 1
Pitfalls
Clinical manifestations overlap with those of many other diseases that present with signs of
vasculitis; consider Kawasaki disease in any child presenting with prolonged, unexplained fever
to ensure that the diagnosis is not missed
Kawasaki disease should be on the differential list for any child with unexplained fever
lasting more than 5 days
Symptoms can be sequential rather than simultaneous, and not all physical examination
criteria may be seen at time of presentation; therefore, a detailed and accurate history is
essential to ensure that the diagnosis is not missed
Inflammatory marker levels (eg, C-reactive protein level, erythrocyte sedimentation rate, WBC
count) may not be abnormal at presentation, and serial measurements may be needed to
confirm the diagnosis when Kawasaki disease is incomplete
Young infants may present with fever and few other principal clinical features; consider
echocardiography in any infant younger than 6 months who is exhibiting fever for 7 or more
days and has laboratory evidence of systemic inflammation and no other explanation for febrile
illness 1
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Because treatment is typically delayed, patients with incomplete Kawasaki disease are at
greatest risk for coronary artery aneurysms, so a high index of suspicion is warranted
Terminology
Clinical Clarification
Kawasaki disease is an acute, self-limited, acquired inflammatory disorder characterized by
vasculitis of small to medium vessels that can lead to coronary artery aneurysms, usually in
children
Classification
Complete Kawasaki disease 1
Fever for 5 or more days, 4 of 5 key diagnostic criteria are met, and absence of viral or
bacterial causes for symptoms. The 5 key criteria are as follows:
Rash: maculopapular, diffuse erythroderma, or erythema multiforme–like
Erythema and cracking of lips, strawberry tongue, and/or erythema of oral and pharyngeal
mucosa
Bilateral bulbar conjunctival injection without exudate
Erythema and edema of hands and feet in acute phase and/or periungual desquamation in
subacute phase
Cervical lymphadenopathy (1.5 cm or more in diameter); typically unilateral
Incomplete Kawasaki disease 1
Fever for 5 or more days and failure to meet at least 4 of 5 diagnostic criteria for complete
Kawasaki disease
Represents 15% to 20% of Kawasaki disease diagnoses 2
Classification by disease phase 3
Esquema Acute: up to day 10

Fever begins and persists; onset of skin, lip, mucous membrane, conjunctival, extremity,
lymph node, and joint involvement
Gradual onset of myocarditis, with peak involvement at day 7, then tapering off by day 14
Subacute: day 10 to week 6.5
Fever can persist without effective treatment through subacute phase; skin, lip, mucous
membrane, conjunctival, extremity, lymph node, and joint involvement gradually resolve
Aneurysms usually start to develop around week 3, with gradual decrease in aneurysm
formation and inflammation by end of subacute phase
Peak inflammatory changes are noted around week 5
Risk for sudden death is highest in this phase
Convalescent: weeks 6.5 to 9
Resolution of fever and other acute inflammatory changes
Unusual to detect new aneurysm formation after week 8 of illness
Diagnosis
Clinical Presentation
History
Symptom manifestation often occurs sequentially rather than
simultaneously 4
Some clinical features may have resolved by time of Kawasaki disease: conjunctivitis. -
presentation, whereas others evolve after initial Nonexudative, nonulcerative, bulbar
presentation conjunctivitis. Note sparing of the
limbus.
Mild, nonspecific upper respiratory tract or
gastrointestinal tract symptom prodrome is common
Fever for 5 or more days 5 is present in 100% of cases

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Fever temperatures are generally high (higher than 39 °C
and commonly higher than 40 °C)
Defervescence is usually delayed until acute inflammatory

phase resolves. If disease is untreated, fever may persist for
up to 6 weeks
Fever is minimally responsive to antipyretics 6

Kawasaki disease: oral findings. - A,
Extreme irritability is very common and may precede
Erythematous, cracked lips. B,
development of fever Strawberry tongue.
General fussiness
Paroxysmal irritability
Rash
Generalized rash is usually reported, lasting up to 2 weeks 3
Lips and oral cavity

Kawasaki disease: truncal rash. -
Red, cracked, dry, painful lips: symptoms are noted about Morbilliform rash is one possible
day 2 or 3 and peak around day 7 3 manifestation.
Red mouth, tongue swelling, and sore throat occur in same

time frame
Eyes Kawasaki disease: perineal rash and

neck rash. - A, Perineal rash with
Ocular symptoms usually appear shortly after onset of fever peeling. B, Neck rash with peeling.
and peak around day 7 3 Note that peeling of an
intertriginous rash occurs before
Red, irritated eyes without discharge extremity peeling.
Photophobia and eye pain
Blurry vision
Extremities
Kawasaki disease: swollen,
Symptoms begin around day 4, peak at about day 10, and
erythematous hands. - Note the
resolve by 2 or 3 weeks 3 fusiform appearance.
Esquema Swelling of hands and feet

Reddening of skin on hands and feet, especially palms and
soles, with later peeling of skin on fingers and toes
Pain or refusal to bear weight or ambulate
Neck swelling from lymphadenopathy begins at about day 5,

peaks at day 14, and resolves by 2 or 3 weeks 3 Kawasaki disease: fingertip and toe
tip peeling. - Subacute phase of
disease.
Joint pain and swelling are usually mild in first few days of
illness, but with worsening symptoms through days 14 to 16,
then gradual improvement by week 3 or 4 3
Gastrointestinal symptoms
Vomiting is a common nonspecific symptom
Diarrhea and abdominal pain may be reported Kawasaki disease: Beau lines of
fingernails. - Convalescent phase of
Less commonly, rhinorrhea and cough are reported in early disease.
stage of disease
Physical examination
Polymorphous rash typically develops early in course of
illness and is present in about 80% to 90% of cases 6 7
Many types of rashes are described: maculopapular,

scarlatiniform, urticarial, erythema multiforme–like,
micropustular, diffuse erythroderma, and/or scaling Kawasaki disease: inflammation of
urethral meatus. - Often associated
plaques with sterile pyuria.
Almost never bullous or vesicular
Usually extensive distribution; accentuated on trunk,

extremities, and perineal area
Perianal erythema in 67% of cases 7

Kawasaki disease: inflammation of
Erythema and induration at a site of previous BCG vaccine face and neck. - After treatment with
inoculation is common in children born in countries IV immunoglobulin, the patient's
where the vaccine is used widely (bacille Calmette-Guérin) 1 lymphadenopathy resolved and the
facial edema improved back to
Oropharyngeal mucous membrane changes are present in baseline.
80% to 90% of cases 6 7
Esquema
Dry, cracked, erythematous lips
Swollen strawberry tongue
Nonexudative erythema of posterior oropharynx
Marked erythema of oropharyngeal mucosa
Conjunctival injection is present in 80% to 90% of cases 6 7
Typically the conjunctivitis is bilateral, painless, and not associated with exudate
Classically described as limbic-sparing injection of bulbar conjunctiva
Ocular examination shows anterior uveitis or acute iridocyclitis in up to 83% 8
Decreased visual acuity
Photophobia
Perilimbal injection
Pupillary miosis
Abnormal slit lamp examination findings, with endothelial precipitates and hazy aqueous
humor
Extremity changes are present in 80% to 90% of cases 6 7
Can be subtle, with bilateral edema and/or erythema of palms and soles lasting 1 to 3 days 9
May progress to involve entire feet and hands, with sharp demarcation of erythema at ankle
and wrist
Extremity changes are usually the last to develop of the 5 primary symptoms 6
Sheetlike desquamation of fingers and toes is often seen late in course (2 to 3 weeks)
Desquamation usually begins in periungual region of fingers and toes
Beau lines are deep transverse grooves across nails that may develop 1 to 2 months after
onset of fever 1
Lymphadenopathy is present in about 50% of cases 6 7
Esquema
Usually a single lymph node, unilateral, anterior cervical, firm, nonfluctuant, and more than
1.5 cm in diameter
Diffuse lymphadenopathy is inconsistent with diagnosis
Cardiac examination findings are usually abnormal, reflecting myocarditis (nearly universal),
pericarditis, endocarditis, and valvular and coronary artery involvement 1
Tachycardia
Hyperdynamic precordium
Gallop 10
Rubs
New murmur
Muffled heart tones
Signs of aseptic meningitis in 50% 11
Guarding of movements
Mild nuchal rigidity
Positive Kernig and Brudzinski signs
Other common physical examination findings include the following:
Erythema and induration at site of previous BCG vaccine inoculation (bacille Calmette-
Guérin)
Oligoarticular or polyarticular arthritis/arthralgias in 7.5% to 30% 7
Swollen, red, tender joints
Most commonly affects lower extremities
Small joint polyarthritis is typically observed in the first week
After 1 to 2 weeks, large joint pauciarthritis is typical
Signs of gallbladder hydrops 1

Esquema
Right upper quadrant pain
Jaundice
Other less common findings include the following:
Shock (usually cardiogenic)
Thready pulses, prolonged capillary refill, tachycardia, and hypotension late
Murmur and/or gallop
Hepatomegaly and jaundice
Signs of peripheral facial nerve palsy
Hemifacial paresis of upper and lower face
Signs of encephalopathy
Waxing and waning mental status changes and confusion
Ataxia
Causes and Risk Factors
Causes
No known causes 6
Many studies have attempted to identify a causative agent, but no known viral, bacterial,
rickettsial, spirochetal, fungal, or parasitic agent has been definitively identified
No conclusive environmental trigger has been identified
May be due to an immune response to 1 or more infectious antigens 12
Autoimmunity may be part of the immunologic process 9
Clinically apparent disease may appear in genetically susceptible persons who mount a
deleterious host response 10
Sometimes called Kawasaki syndrome in recognition of nosologic unknowns

Esquema
Risk factors and/or associations
Age
Typically 6 months to 5 years 13
85% of affected children are younger than 5 years 14
Disease is rare in adults 15
Disease occurs less commonly in children younger than 6 months or older than 5 years, but
these age groups are at higher risk for development of coronary artery aneurysms 14
Children younger than 6 months or older than 5 years tend to have more severe disease
Incomplete disease also tends to occur at the extremes of this age spectrum (either younger
than 1 year or older than 5 years) 16
Sex
Incidence is higher in boys than in girls (1.5:1) 3
Genetics
No clear pattern of inheritance
Clusters of disease have been reported in families 17
Children whose parents had Kawasaki disease are at slightly increased risk 18
Siblings of children with disease are at 10 times increased risk 19
Several genetic associations are linked with disease, as follows:
MICA alleles (major histocompatibility complex class I polypeptide–related sequence A) 20
Increased frequency of allele *A5
Decreased frequency of alleles *A5.1 and *A4
HLA class I gene sequence variants 20

Esquema
Increased frequency of HLA-B35, HLA-B75, HLA-Cw09, HLA-DRB1*11, and HLA-
DRB1*04
Decreased frequency of HLA-A26
ITPKC (inositol 1,4,5-triphosphate 3-kinase C), single nucleotide variant 20
Increased frequency of itpkc_3
Other identified abnormalities (eg, single nucleotide variants) have been found in various
susceptibility genes, including:
BLK (B lymphoid tyrosine kinase) 9
CASP3 (caspase 3) 9
FCGR2A (low-affinity immunoglobulin gamma Fc region receptor IIa) 9
CD40 signaling pathway genes 9
Transforming growth factor and receptor genes (eg, TGFB2, TGFBR2, SMAD3) 9
ABCC4 (ATP binding cassette subfamily C member 4, a cyclic nucleotide transporter) 21
ANGPT1 (angiopoietin 1) 22
VEGFA (vascular endothelial growth factor A) 22
Ethnicity/race
Incidence in people of northeast Asian descent is up to 20 times higher than in white
populations 9
Incidence is highest among Japanese populations and Pacific Islanders 1
Other risk factors/associations

Most common in winter and spring 23
Epidemiologic analyses have correlated the incidence of cases in Japan, Hawaii, and San Diego
with tropospheric wind currents originating in northeastern China, suggesting that a wind-
borne agent might trigger the illness 1
Esquema
Diagnostic Procedures
Primary diagnostic tools

Symptoms can be sequential rather than simultaneous, and not all physical examination
criteria may be seen at time of presentation; therefore, a detailed and accurate history is
essential to ensure that the diagnosis is not missed
When the diagnostic criteria for complete Kawasaki disease are met, the diagnosis is based
on history and physical examination alone 1
CBC, erythrocyte sedimentation rate, and C-reactive protein measurement are routine at
initial presentation and are used to follow up for resolution of inflammation and
efficacy of treatment
If clinically indicated, obtain basic metabolic profile, liver function tests, cerebrospinal
fluid analysis, and urinalysis
Obtain baseline ECG and echocardiogram in all patients to assess degree of cardiac
involvement
Other tests to investigate for alternate diagnosis may be indicated based on clinical
presentation (eg, rapid viral testing, blood culture, specific serology for suspected
infections)
Positive result of polymerase chain reaction assay for viral pathogen (eg, rhinovirus,
enterovirus) does not fully preclude the diagnosis of Kawasaki disease 24
Diagnosis of incomplete Kawasaki disease
Dangerous diagnostic dilemma; seek consultation with an expert (usually pediatric

cardiologist or pediatric infectious disease specialist) any time assistance is needed 1
Because treatment is typically delayed, patients with incomplete Kawasaki disease are
at greatest risk for coronary artery aneurysms, so a high index of suspicion is
warranted
Most common findings in children are cervical lymphadenopathy (usually unilateral)

and extremity changes (although frequency of finding these in incomplete disease is
somewhat lower than in complete disease) 16
Other subtle findings that increase suspicion include inflammation at site of BCG
Esquema vaccine inoculation (bacille Calmette-Guérin), gall bladder hydrops, sterile cerebrospinal
fluid pleocytosis, elevated levels of B-type natriuretic peptide and N-terminal pro–B-
type natriuretic peptide, hyponatremia, elevation of the left ventricular mass, and left
ventricular diastolic dysfunction 16
Guidelines suggest that further laboratory testing is indicated for both of the following
groups: children with fever lasting 5 or more days who have 2 or 3 typical characteristics
of Kawasaki disease and infants with fever lasting 7 or more days without other
explanation 1
When patient has C-reactive protein of 3.0 mg/dL or higher and/or erythrocyte
sedimentation rate of 40 mm/hour or higher: 1
If 3 or more of the following supplemental laboratory test results are positive or

patient has a positive echocardiogram, begin treatment: 1
Anemia for age 1
Platelet count of 450,000 cells/mm³ or more (after day 7 of fever) 1
Albumin level of 3 g/dL or less 1
Elevated ALT level 1
WBC count of 15,000 cells/mm³ or more 1
Presence of pyuria (urine has 10 or more WBCs per high-power field) 1
Echocardiogram is considered positive if any of the following conditions are met: 1
Z score of left anterior descending coronary artery or right coronary artery of 2.5
or higher 1
Coronary artery aneurysm is observed 1
3 or more other suggestive features exist, including the following: 1
Decreased left ventricular function
Mitral regurgitation
Pericardial effusion
Z scores in left anterior descending coronary artery or right coronary artery of

2 to 2.5
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If C-reactive protein is less than 3.0 mg/dL and erythrocyte sedimentation rate is less
than 40 mm/hour: 1
Serial clinical and laboratory re-evaluation if fevers persist 1
Echocardiogram if typical peeling develops, which begins under nail beds 1
Other promising, supplemental biomarker abnormalities may help with diagnostic

suspicion for acute phase disease, including:
Elevated levels of N-terminal pro–B-type natriuretic peptide, apolipoprotein B,

haptoglobin, creatine kinase MB, and serum cardiac troponin I; expression of inducible
nitric oxide synthase in neutrophils; and elevated levels of fibrinogen-related plasma
protein 20
Diminished levels of apolipoproteins A to I 20
Presence of associated genetic associations and HLA variants 20
Other laboratory abnormalities that may be present include:
Hyponatremia (sodium less than 135 mEq/L) 25
May be a risk for giant coronary artery aneurysms
Abnormal serum lipid profiles (eg, elevated triglyceride levels, decreased HDL and total
cholesterol levels) 26
Detailed guidelines to assist in the diagnostic process are available as follows:
Japanese Circulation Society Joint Working Group 2020 guidelines 27
Japanese Society of Pediatric Cardiology and Cardiac Surgery 2012 guidelines 28
Italian Society of Pediatrics 2018 guidelines 29
American Heart Association 2017 guidelines 1
Laboratory
Imaging
Esquema
Functional testing
Procedures
Other diagnostic tools
Differential Diagnosis
Most common
General Clinical manifestations overlap with those of many other
diseases that present with signs of vasculitis; consider
Kawasaki disease in any child presenting with prolonged,
unexplained fever to ensure that the diagnosis is not missed
Kawasaki disease should be on the differential list for any

child with unexplained fever lasting more than 5 days
Young infants may present with fever and few other principal
clinical features; consider echocardiography in any infant
younger than 6 months who is exhibiting fever for 7 or more
days and has laboratory evidence of systemic inflammation
and no other explanation for febrile illness 1
Adenovirus (Related: Presents with prominent ocular erythema and drainage,

Adenovirus infections) coryza, fever, and sore throat
Not associated with rash, lip, or extremity changes
Typically lasts for 3 to 5 days
Significant elevations in erythrocyte sedimentation rate and

C-reactive protein level are not characteristic
Differentiated with polymerase chain reaction analysis to

identify adenovirus
Esquema
Enterovirus Enterovirus disease is broad and is most often associated
with an undifferentiated febrile illness with fevers lasting up
to 1 week
Myopericarditis and aseptic meningitis are associated with

some enteroviral illnesses
Oral lesions typical of hand-foot-and-mouth disease may be

present and are vesicular in nature
Rash associated with enterovirus is more vesicular and is

located mainly on palms, on soles, and in diaper area
Extremity changes and ocular involvement are unusual

Differentiated with polymerase chain reaction assay

demonstrating virus in cerebrospinal fluid
Epstein-Barr virus (Related: Fever in children with Epstein-Barr virus infection tends to
Epstein-Barr Virus be low grade but can persist for weeks
Infection)
Children exhibit more prominent fatigue with Epstein-Barr
virus, with less irritability than seen in Kawasaki disease
Extremity, lip, and ocular changes are not noted in Epstein-

Barr virus infections
Diffuse impressive cervical lymphadenopathy and exudative

pharyngitis with palatal petechiae are more characteristic of
Epstein-Barr virus infection
Splenomegaly is common
WBC count shows lymphocytosis with atypical lymphocytes

Monospot test or Epstein-Barr virus titer is diagnostic

Esquema
Scarlet fever 37 Rash, fever, and lymphadenopathy are present
Lip changes, ocular changes, and extremity changes are not

present
Positive rapid streptococcal test or culture result is diagnostic
Rheumatic fever (Related: Tends to develop in children older than 5 years

Rheumatic Fever)
Jones criteria are helpful in differentiating it from Kawasaki
disease 38
Results are positive from streptococcal antibody tests, rapid

antigen tests, or throat culture for group A β-hemolytic
streptococcus
Bacterial toxin–mediated Staphylococcal scalded skin syndrome

illness
Typically severe blistering rash is present, with subsequent
sheet-like epidermal shedding
Spares the mucous membranes; articular signs are

generally absent
Skin biopsy can confirm this diagnosis
Culture from nares or wound with result positive for

toxin-producing Staphylococcus aureus is diagnostic
Toxic shock syndrome (Related: Toxic Shock Syndrome)
History of retained foreign body, such as tampon or nasal

packing, is usual
Hypotension, thrombocytopenia, central nervous system

involvement (eg, confusion), and renal failure are common
Edema is generally diffuse and not limited to hands and

feet; articular signs are generally absent
Case definition involves isolation of group A

Esquema
streptococcus, hypotension, and multisystem involvement
Bacterial cervical Cases of incomplete Kawasaki disease in older children have
lymphadenitis been described as presenting initially with only fever and
cervical lymphadenopathy
Cardiac and ocular involvement that is typically seen with

Kawasaki disease is absent in children with bacterial
lymphadenitis
Children with cervical lymphadenitis may have pain with

neck movement, but they do not have true signs of central
nervous system irritability or nuchal rigidity as are observed
in some cases of Kawasaki disease
C-reactive protein level, erythrocyte sedimentation rate, and

platelet count are not elevated significantly in these patients
RBC indices, liver function test results, and urinalysis results

should be normal in children with cervical lymphadenitis
Drug hypersensitivity Serum sickness

syndromes
History of drug or antitoxin/antisera/antivenom exposure
1 to 2 weeks prior
Inflammatory marker levels are only slightly elevated

compared with levels found in Kawasaki disease
Complement levels are low, and eosinophilia may be

present
Stevens-Johnson syndrome (Related: Stevens-Johnson

Syndrome and Toxic Epidermal Necrolysis)
History of drug exposure, prior infection, or malignancy
Rash progresses to bullae that rupture, leaving denuded

skin that resembles a burn
Hypotension can be seen
Esquema Skin biopsy can be helpful if diagnosis is in question

Juvenile rheumatoid May be difficult to distinguish from acute phase of Kawasaki
arthritis ( juvenile disease until chronicity of symptoms and prominent
idiopathic arthritis) polyarteritis are evident
(Related: Rheumatoid
Arthritis) Most cases have a positive antinuclear antibody test result
Oropharyngeal, conjunctival, and extremity changes typical

with Kawasaki disease are not usually associated with juvenile
rheumatoid arthritis
Lymphadenopathy is often generalized and associated with

splenomegaly in patients with juvenile rheumatoid arthritis
Systemic lupus Children tend to have significant renal, central nervous

erythematosus 39 (Related: system, and hematologic involvement, along with
Systemic Lupus panserositis involving heart and lungs
Erythematosus)
More of an insidious onset, with fevers, malaise, joint pain,
and rash
Rash is usually photosensitive; malar rash is characteristic
Oral ulcers are typical
Pancytopenia
Proteinuria is frequent
Low complement levels
Positive direct Coombs test result in absence of hemolytic

anemia
Positive results for antinuclear, anti–double-stranded DNA,

anti-Sm, or antiphospholipid antibodies (including lupus
anticoagulant); presence of antinuclear antibody is the
cardinal feature, positive in virtually all patients
Base diagnosis on a constellation of characteristic symptoms,

signs, and laboratory findings in the appropriate clinical
context
Esquema
Leukemia, lymphoma, or Timeline of symptoms is chronic
other neoplasm
Bleeding
Bruising
Bone pain
Hepatosplenomegaly and lymphadenopathy are

characteristic
Profound changes in CBC are typical
Very low or high WBC counts
Significant anemia
Thrombocytopenia
Bone marrow biopsy or lymph node biopsy is diagnostic in

most cases
Rubeola Fever, cough, conjunctivitis, and generalized erythematous

maculopapular rash occur
Rash begins as exanthem on face and neck and spreads

distally to trunk and extremities
Koplik spots (nontender bluish white lesions on an

erythematous base) are pathognomonic, appearing on buccal
mucosa near second molars
Suspicion is higher in settings of known outbreak or

exposure, in persons returning from travel to endemic areas,
and in persons who have not been immunized
IgM antibody testing can confirm infection
Rocky Mountain spotted High fever, severe headache, rash, thrombocytopenia,

fever (Related: Rocky hyponatremia, and tick exposure history
Mountain Spotted Fever)
Esquema
Rash begins as blanchable erythematous macules on
extremities; it evolves to maculopapular rash, then petechiae,
while spreading centrally to trunk
Result of immunofluorescence assay or skin biopsy should

confirm diagnosis
Leptospirosis Zoonotic infectious disease caused by pathogenic serotypes

of the bacterial spirochete Leptospira; liver and kidney are
major target organs and disease severity ranges from mild
and self-limiting to life-threatening multisystem organ
dysfunction 40
Endemic areas include tropical warm climates; seasonal

epidemics occur after heavy rainfall or flooding in rainy
season 40
Most cases present as mild, self-limiting, nonspecific febrile

illness; a minority are severe and result in jaundice with
hepatic dysfunction, renal insufficiency, and bleeding
diathesis
Rash is uncommon 40
Differentiated by confirming infection with detection of

leptospira antibodies; a combination of tests is sometimes
necessary to confirm diagnosis owing to narrow windows of
positivity for individual testing methods 41
Treatment
Goals
Reduce fever 10 and acute inflammatory changes
Reduce platelet activation
Esquema
Prevent potential cardiac sequelae
Disposition
Admission criteria
Admit all children with Kawasaki disease (complete or incomplete) to a pediatric inpatient facility
Criteria for ICU admission

Clinical suspicion of myocarditis
Impaired ventricular function
Heart failure
Shock
Giant coronary aneurysms seen on echocardiogram
Defined as maximum diameter of greater than 8 mm 1
Ischemic heart disease as indicated on ECG by ST-T wave changes or Q waves
Recommendations for specialist referral

Urgently refer all children with Kawasaki disease to a pediatric cardiologist for both evaluation
and follow-up
Treatment Options
Primary treatment for Kawasaki disease is IV immunoglobulin and high-dose aspirin 42 43
Treatment should begin promptly (to minimize risk of coronary artery aneurysm formation)
when the following occur: 10
Criteria have been met for complete Kawasaki disease (even if present before day 5 of fever)
Concern persists after day 5 of fever for incomplete Kawasaki disease, and 1 or both of the
following occur:
Coronary artery aneurysms or signs of coronary arteritis are present

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Persistent elevation of inflammatory marker levels exists, with no other explanation (eg,
increased C-reactive protein level, increased erythrocyte sedimentation rate, leukocytosis)
20% of patients will not have response to initial IV immunoglobulin and aspirin treatment 10
11 44
IV immunoglobulin resistance is defined as persistent or recurrent fever at least 36 hours

after first infusion dose 4
Corticosteroids may be added to the primary IV immunoglobulin and aspirin treatment at the
recommendation of a pediatric cardiologist in patients at high risk for IV immunoglobulin
resistance and development of coronary artery aneurysms 10 45 46 47
IV immunoglobulin plus corticosteroid added to the initial treatment may improve clinical
and coronary outcomes for patients predicted to be at high risk for IV immunoglobulin
resistance and for those with coronary artery aneurysms present at time of diagnosis 28
Several scoring systems are available to help predict resistance; 28 however, these scoring
systems are not validated and may have limited use outside initial study population 48
Treatment for IV immunoglobulin resistance
Administer a second dose with or without corticosteroid 10 28
IV immunoglobulin retreatment is effective in up to half of patients with resistance to first

infusion 28
Other second line treatments are as follows (optimal choice and sequence are not known):
tumor necrosis factor blockade (eg, infliximab, etanercept) 49, immunosuppressants (eg,
cyclosporin A, methotrexate), and plasma exchange 28
Management of subacute and convalescent phases with expert advice is as follows: 10
Treatment decisions should be made in consultation with a pediatric cardiologist
Dose of aspirin is decreased to 3 to 5 mg/kg for 7 weeks or more, until findings on follow-up
echocardiograms are normal
Continued antiplatelet therapy with low-dose aspirin or clopidogrel is indicated if aneurysms

persist after that time
Antiplatelet therapy can be discontinued if aneurysms resolve
Abciximab may be considered in patients with coronary artery aneurysms

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Warfarin in addition to aspirin is indicated in children who develop giant aneurysms, multiple
large aneurysms, or signs of coronary artery obstruction 4
Heparin therapy is started until warfarin is therapeutic as indicated by goal INR of 2 to 3
β-blockers are often added to reduce myocardial oxygen consumption if signs of coronary
artery obstruction develop 4
Thrombolytic therapy is started if coronary artery thrombosis occurs
Management of acute phase aneurysms is not standardized and must be individualized in

consultation with a pediatric cardiologist
Long-term monitoring with regular echocardiography, stress tests, and cardiac perfusion tests
is necessary 4
Patients at high risk of ischemia are candidates for percutaneous coronary intervention
procedures (eg, intracoronary thrombolysis, balloon angioplasty, stent implantation, rotational
ablation) 4
Patients with severely occlusive lesions or jeopardized collateral blood supply require coronary
artery bypass surgery 4
Emergent coronary angioplasty or other revascularization procedure is indicated if obstruction

or ischemia develop 1
Immunizations after Kawasaki disease
Delay immunization by 3 to 11 months after IV immunoglobulin treatment, because live

vaccines (eg, rubeola, varicella-zoster) may be potentially ineffective 50
Caution is warranted when live varicella-zoster vaccine is administered in patients receiving

long-term aspirin therapy, owing to theoretical concern for development of Reye syndrome 1
In children taking long-term salicylates, provide annual inactivated influenza vaccine 1
Detailed guidelines to assist in the treatment process are available
Japanese Circulation Society Joint Working Group 2020 guidelines 27
Japanese Society of Pediatric Cardiology and Cardiac Surgery 2012 guidelines 28
Italian Society of Pediatrics 2018 guidelines 29
American Heart Association 2017 guidelines 1

Esquema
Drug therapy
Aspirin 42 51
Off-label use
Aspirin Chewable tablet; Infants, Children, and Adolescents: 80 to 100 mg/kg/day PO in 4

divided doses during the acute phase (often until patient has been afebrile for 24 to 72
hours, for up to 14 days), then decrease to 3 to 5 mg/kg/day PO once daily (Max: 325
mg/day) until 4 to 6 weeks after the onset of illness. For those who develop coronary
abnormalities, low-dose aspirin may continue indefinitely.
IV immunoglobulin 43 52
Immune Globulin (Human) Solution for injection; Infants, Children, and Adolescents: 2,000
mg/kg IV single dose within 10 days of illness but as soon as possible after diagnosis. Treat
patients after day 10 if they have unexplained persistent fever or coronary artery
abnormalities with ongoing systemic inflammation. Consider second dose (2,000 mg/kg) if
fevers persist or reappear at least 36 hours after completion of initial IVIG infusion.
Immune Globulin (Human) Solution for injection; Infants, Children, and Adolescents: 1,000
mg/kg IV as a single dose or 400 mg/kg/dose IV daily for 4 days beginning within 7 days of
fever onset.
Corticosteroids 1 53
Off-label use
Both prednisolone and high-dose methylprednisolone pulse dosing have been used 28
Corticosteroids may be indicated in patients at high risk for developing coronary artery
aneurysms and in IV immunoglobulin–resistant disease. 46 54 45 Select dose and duration of
therapy in consultation with a specialist
Infliximab (anti–tumor necrosis factor α) 10
Off-label use
Infliximab may be indicated in IV immunoglobulin–resistant disease. 55 56 Select dose and

duration of therapy in consultation with a specialist
Abciximab
Esquema Off-label use

Abciximab may be indicated as adjunct treatment in children who develop aneurysms. 57
Select dose and duration of therapy in consultation with a specialist
Clopidogrel
Off-label use
Clopidogrel may be indicated as adjunct treatment. Select dose and duration of therapy in
consultation with a specialist 1
Etanercept 1
Off-label use
May be considered as a second-line agent in IV immunoglobulin–resistant disease. 1 Select

dose and duration of therapy in consultation with a specialist
Nondrug and supportive care

Advise parents that children who have experienced Kawasaki disease with acute phase coronary
artery involvement must reduce or avoid exacerbating risk factors for atherosclerosis (eg,
obesity, hyperlipidemia, smoking) 4
Monitoring
Monitoring during treatment
Monitor clinically for fever after IV immunoglobulin treatment
Nonresolution of fever in the hospital or return of fever in the first few days after treatment
signify need to carefully evaluate for recrudescent disease
Recommended echocardiogram monitoring schedule 10
Acute, subacute, and convalescent phases without established aneurysms
At time of diagnosis
10 to 14 days after onset of disease
6 to 8 weeks after onset of disease
In patients with ongoing active inflammation, obtain weekly echocardiograms

Esquema
If during course of Kawasaki disease the findings on all other repeated echocardiograms
are normal, 1 repeated echocardiogram is indicated at 1 year remote from acute disease
With aneurysms
Monitor evolving or established aneurysms with weekly echocardiograms
In patients with stable aneurysms and no ongoing inflammation after 8 weeks, obtain
echocardiograms every 6 to 12 months
Long-term cardiovascular follow-up 1 is indicated in children with persistent aneurysms

beyond 6 weeks after diagnosis
Detailed guidance and long-term recommendations for follow-up are available in an

American Heart Association statement. 1 These recommendations are based on stratification
of risk for future development of myocardial ischemia
Complications and Prognosis
Complications
Coronary artery aneurysms secondary to coronary artery vasculitis
Occur in 15% to 25% of untreated children 44
2% to 3% of untreated patients die of untreated coronary vasculitis 44
Occur in less than 5% if treatment is begun within 10 days of fever onset 58
3% to 5% of children develop transient coronary artery dilation despite appropriate

treatment 4
1% will develop giant coronary artery aneurysm despite appropriate treatment 59
Risk factors for coronary artery aneurysm development include:
IV immunoglobulin resistance (strongest risk factor) 10
Late IV immunoglobulin treatment (after 7 or more days of illness) 60 and longer duration
of fever before treatment 4
Esquema
Incomplete Kawasaki disease (likely owing to higher risk for delay in diagnosis and
treatment) 60
Age younger than 1 year or older than 5 years 4
Possibly male sex, 61 hyponatremia at presentation, 25 or predisposed ethnicity (eg, Asian,

Pacific Islander, Hispanic) 62
Increasingly severe degrees of thrombocytosis and leukocytosis 63
Myocardial infarction
Peak mortality is seen at 15 to 45 days after fever onset; death is usually caused by coronary
artery thrombosis and ischemia 58
Coronary vasculitis occurs concomitantly with marked elevation in platelet count and
related hypercoagulable state
Myocardial infarction from thrombotic vessel occlusion or stenosis is the highest risk in the
first year after diagnosis 64
Noncoronary vascular involvement
Medium-sized muscular arteries (eg, axillary, iliac, brachial, mesenteric) are most commonly
affected
Rarely results in clinical ischemic sequelae involving central nervous system or renal,
gastrointestinal, or cutaneous organ systems
Macrophage activation syndrome (secondary hemophagocytic lymphohistiocytosis)
Results in cytopenias, hepatosplenomegaly, hypofibrinogenemia, and liver dysfunction
Rarely occurs (less than 1%) in children with disease resistant to IV immunoglobulin and in
those with very prolonged fever 65
Death
Risk for sudden death is highest in subacute phase and in children younger than 1 year
Prognosis
Prognosis in children is largely determined by degree of cardiac involvement and potential
cardiac sequelae
Esquema
Children without known cardiac sequelae during the first month of Kawasaki disease appear to
return to their baseline state of health without signs or symptoms of cardiac impairment 58
Coronary artery lesions resulting from Kawasaki disease change dynamically with time 58
Resolution of aneurysms 1 to 2 years after onset of disease is observed in about 50% to 67%
of vessels 58
Likelihood of aneurysm resolution depends on initial size; smaller lesions are more likely to
regress 58
Worst prognosis occurs in children with giant aneurysms 66 (bigger than 8 mm in diameter),
which tend to thrombose and never resolve completely
Children with regression of aneurysms from Kawasaki disease are still at increased risk of
developing early-onset coronary artery disease as adults
Case fatality rate is less than 0.1% in Japan 1
In-hospital mortality rate in the United States is about 0.17% 1
Sudden death from myocardial infarction may occur many years later in people who as
children had coronary artery aneurysms and/or stenoses 58
Recurrence
Recurrence rates are low (2%-4%); recurrence is more common in children younger than 3
years at first diagnosis 64
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Key Points Urgent Action

In patients with Kawasaki disease with aneurysms on echocardiogram, ongoing monitoring

Rash: maculopapular, diffuse erythroderma, or erythema multiforme–like

Bilateral bulbar conjunctival injection without exudate

Cervical lymphadenopathy (1.5 cm or more in diameter); typically unilateral

Incomplete Kawasaki disease 1

Represents 15% to 20% of Kawasaki disease diagnoses 2

Classification by disease phase 3

Esquema Acute: up to day 10

Subacute: day 10 to week 6.5

Peak inflammatory changes are noted around week 5

Risk for sudden death is highest in this phase

Convalescent: weeks 6.5 to 9

Resolution of fever and other acute inflammatory changes

Unusual to detect new aneurysm formation after week 8 of illness

Fever for 5 or more days 5 is present in 100% of cases

Defervescence is usually delayed until acute inflammatory

Fever is minimally responsive to antipyretics 6

Generalized rash is usually reported, lasting up to 2 weeks 3

Lips and oral cavity

Red mouth, tongue swelling, and sore throat occur in same

Eyes Kawasaki disease: perineal rash and

Photophobia and eye pain

Esquema Swelling of hands and feet

Pain or refusal to bear weight or ambulate

Neck swelling from lymphadenopathy begins at about day 5,

Vomiting is a common nonspecific symptom

Many types of rashes are described: maculopapular,

Almost never bullous or vesicular

Usually extensive distribution; accentuated on trunk,

Perianal erythema in 67% of cases 7

Swollen strawberry tongue

Nonexudative erythema of posterior oropharynx

Marked erythema of oropharyngeal mucosa

Conjunctival injection is present in 80% to 90% of cases 6 7

Classically described as limbic-sparing injection of bulbar conjunctiva

Ocular examination shows anterior uveitis or acute iridocyclitis in up to 83% 8

Decreased visual acuity

Extremity changes are present in 80% to 90% of cases 6 7

Desquamation usually begins in periungual region of fingers and toes

Lymphadenopathy is present in about 50% of cases 6 7

Diffuse lymphadenopathy is inconsistent with diagnosis

Muffled heart tones

Signs of aseptic meningitis in 50% 11

Mild nuchal rigidity

Positive Kernig and Brudzinski signs

Other common physical examination findings include the following:

Oligoarticular or polyarticular arthritis/arthralgias in 7.5% to 30% 7

Swollen, red, tender joints

Most commonly affects lower extremities

Small joint polyarthritis is typically observed in the first week

After 1 to 2 weeks, large joint pauciarthritis is typical

Signs of gallbladder hydrops 1

Other less common findings include the following:

Shock (usually cardiogenic)

Thready pulses, prolonged capillary refill, tachycardia, and hypotension late

Murmur and/or gallop

Hepatomegaly and jaundice

Signs of peripheral facial nerve palsy

Hemifacial paresis of upper and lower face

Waxing and waning mental status changes and confusion

Decreased frequency of alleles A5.1 and A4