Kawasaki Disease

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Kawasaki Disease

• KD is a vasculitis of unknown etiology that is
  characterized by multisystem involvement and
  inflammation of small to medium-sized arteries
  with resulting aneurysm formation.
• It was first described in 1967 by Dr. Tomisaku
  Kawasaki in Japan
• EPIDEMIOLOGY
• variable frequency in all parts of the world, the
  highest of which is in Japan (175 per 100,000)
• The incidence in the U.S. is approximately 6 per
  100,000 children younger than age 5 years.
• The disease affects boys more than girls
• KD can affect children of all races, it is more
  common among children of Asian descent.
• ? Seasonal variability
• CLINICAL MANIFESTATIONS
• three phases
• Acute Phase
• Subacute Phase
• Convalescent
  Phase
• Acute Phase
• lasts 1 to 2 weeks, marked by sudden onset of
  a high, hectic fever

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• LABORATORY AND IMAGING STUDIES
• General Exclude other causes of fever!
• Obtain Blood- and Urine Culture
• In the acute phase Inflammatory Parameters are
  elevated ? WBC, ESR, Platelet count (platelets
  are greatly elevated in the subacute phase)
• Specific
• two-dimensional echocardiograms
• usually during the acute phase, at 2 to 3
  
• weeks, and at 6 to 8 weeks
• coronary angiography when Coronary
  abnormalities
• mucosal changes, including dry, cracked lips and
  a strawberry tongue
• Desquamation of the skin, particularly of the
  fingers and toes in the subacute phase
• A rash, which can vary in appearance, occurs in
  80 of children with KD and may be particularly
  accentuated in the inguinal area and on the chest
• The conjunctivitis is bilateral and
  nonsuppurative
• Cervical lymphadenopathy is found in 70 of
  children and should be greater than 1.5 cm in
  diameter for the purposes of diagnosis

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• Complications of Kawasaki Disease
• Coronary artery thrombosis
• Peripheral artery aneurysm
• Coronary artery aneurysms
• Myocardial infarction
• Myopericarditis
• Congestive heart failure
• Hydrops of gallbladder
• Aseptic meningitis
• Irritability
• Arthritis
• Sterile pyuria (urethritis)
• Thombocytosis (late)
• Diarrhea
• Pancreatitis
• Peripheral gangrene
• TREATMENT
• IV immunoglobulin (IVIG) is the mainstay of
  therapy for KD

CLINICAL MANIFESTATIONS Rash Arthritis,
migratory less frequently
gastrointestinal or renal vasculitis
Hallmark of HSP ? palpable purpura occasionally
is associated with ?
encephalopathy, pancreatitis,
and orchitis