Download Kawasaki Disease - LSU School of Medicine

Good Morning 
Morning Report
July 2, 2013
Semantic Qualifiers
Symptoms
Acute /subacute
Chronic
Localized
Diffuse
Single
Multiple
Static
Progressive
Constant
Intermittent
Single Episode
Problem Characteristics
Ill-appearing/
Toxic
Well-appearing/
Non-toxic
Recurrent
Localized problem
Systemic problem
Abrupt
Gradual
Acquired
Congenital
Severe
Mild
New problem
Recurrence of old
problem
Painful
Nonpainful
Bilious
Nonbilious
Sharp/Stabbing
Dull/Vague
Illness Script
 Predisposing Conditions
 Age, gender, preceding events
(trauma, viral illness, etc),
medication use, past medical history
(diagnoses, surgeries, etc)
 Pathophysiological Insult
 What is physically happening in the
body, organisms involved, etc.
 Clinical Manifestations
 Signs and symptoms
 Labs and imaging
Differential Diagnosis**
 What other diagnoses would you consider in a
patient with suspected Kawasaki Disease?
Predisposing Conditions
 Which country has the highest prevalence of Kawasaki Disease?

Japan (10x that of US)
 In the US, which ethnicity is most commonly affected? Least
commonly?


Most common in Asians and Pacific Islanders
Least common in caucasians
 Age
Median = 2yo
 76% of cases in <5yo
 Male:Female = 3:2

 Which seasons are you more likely to see patients present with
KD?

Seasonal peaks in winter and spring
Pathophysiology
 Complete etiology is unknown, but features suggest
an infectious source.
 Generalized vasculitis


Affects all blood vessels throughout the body
Which specific vessels are affected in KD?
 Preferentially
involves the coronary arteries
 Process
 Initial neutrophil influx 
 Large mononuclear cells w/lymphocytes and plasma cells 
 Active inflammation 
 Progressive fibrosis and scar formation
Clinical Manifestations
 You are seeing a patient with multiple days of fever
as well as a rash and some other non-focal
symptoms. How many days of fever must be
present before diagnosing a patient with KD?
 What are the other 5 criteria used to diagnose KD?
Conjunctivitis**
 Bilateral bulbar
injection
 No exudate
 Painless
 Limbic sparing
 Shortly after fever
starts
Rash**
 Various forms
 Nonspecific, diffuse with scattered macules & erythematous papules
 Occasionally scarlatiniform, erythroderma, erythema multiforme,

uriticarial, or a fine micropustular eruption
 Not bullous of vesicular
 Often involves diaper area
 Within 5d of fever
Adenopathy**
 Least common feature
 Anterior cervical triangle
 Usually unilateral
 > 1.5 cm
 Firm, nontender
 No overlying erythema
Strawberry Tongue**
 Changes of the lips and oral cavity
 Strawberry tongue
 Cracked, red, swollen, bleeding lips
 Diffuse erythema of oral mucosa
 Oral ulcers and exudates are not seen
Hands and Feet**
 Erythema of palms and soles
 Firm, sometimes painful induration of the hands and feet
 Later desquamation that usually begins in periungal region
(2-3 weeks after fever onset)
Clinical Manifestations**
“C R A S H”
Other**
 Arthritis/arthralgias that involve multiple joints
 In children, what behavioral complaint do parents often give?

Irritability***
 GI complaints



Diarrhea
Vomiting
Abdominal pain
 Hepatomegaly and jaundice
 What abnormal finding may be seen on abdominal imaging
(esp. RUQ)

Acalculous distension of gallbladder…hydrops of the gallbladder
Labs**
 What would your CBC look like?

Leukocytosis




Majority with WBC > 15,000
Predominance of immature and mature granulocytes
Anemia
Thrombocytosis…with platelet counts 500-1000 x 103
 Elevated ESR (>40 mm/hr) and CRP (>3mg/dL)
 Mild to moderate elevation of LFTs
 Mild hyperbilirubinemia
 What abnormality could you see on the UA and urine culture?


Sterile pyuria…+WBC
Negative cultures
 Aseptic meningitis (if CSF obtained)
Treatment**
 High-dose aspirin (80-100mg/kg/day divided QID)
during acute phase of illness 



3-5mg/kg/day until no evidence of coronary changes by 6-8
weeks
Continued aspirin therapy if coronary changes present
 IVIG
 2g/kg/dose (up to 2-3 doses depending on fever)
 Children treated with IVIG and ASA had faster resolution of

fever and fewer coronary abnormalities than those treated

with ASA alone
 Refractory KD…treatment is controversial
Cardiac Complications**
 Coronary artery aneurysm (identified on echo within 1-
2mo of diagnosis)
20-25% of untreated patients; 5% of treated patients
 Resolution within 1-2 years in approximately 50%

 Myocardial infarction
Principal cause of death
 Most occur within 1 year of disease onset but can occur

years later

 Myocarditis
 Valvulitis
 Pericarditis with effusion
Echocardiogram**
 When should you obtain an echo on patients with
suspected Kawasaki disease?



Obtain on all patients with suspected Kawasaki
At diagnosis
Follow-up…usually at 2 weeks and 6 weeks after diagnosis
Follow-Up
Atypical Kawasaki
Thanks 
 No noon conference today!