Kawasaki Disease - Quick Consult
Last Updated / Reviewed: June 2022

Definition
Diagnostic Criteria
Key History
Key Physical Exam
Risk Factors for Kawasaki Disease
Differential Diagnosis
Diagnostic Testing
Clinical Risk and Safety Pearls
Treatment 
Complications

Definition

Kawasaki disease is an acute, self-limited, multisystem vasculitis with unknown etiology diagnosed clinically in children with fever. The most significant vasculitis involves the coronary arteries, which are involved in about 20% of patients. No one single microbial agent or environmental toxin has been proven as causative. The etiology is unknown, but the epidemiology and clinical picture suggest an inciting infection with an abnormal immune response.

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Diagnostic Criteria

Clinical criteria have been established for the diagnosis:

Diagnostic criteria for Kawasaki disease has been established. They include fever of higher than 38.5ºC (103.1ºF) for at least 5 days and 4 of the following 5 criteria are noted:

  • Bilateral nonexudative conjunctival injection
  • Changes in lips, tongue, or oral mucosa (injection drying, fissuring, red strawberry tongue)
  • Changes in peripheral extremities (edema, erythema, desquamation)
  • Rash, primarily on the trunk. May be maculopapular, scarlatiniform, or Erythema multiforme-like.
  • Cervical lymphadenopathy (at least one node greater than or equal to 1.5 cm in diameter)

These criteria are not perfect, with less than 100% sensitivity and specificity. Children who do not meet all the criteria may have an atypical or incomplete case of KS. Also, children who meet all the criteria may have another diagnosis.

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Key History

  • Begins abruptly, lasts an average of 11 days. Acute phase days 1 – 11.
  • Fever: typically lasting at least 5 days, remittent, > 38.5ºC (103.1ºF)
  • Irritability
  • Lethargy
  • Intermittent colicky abdominal pain
  • Rash
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Key Physical Exam

  • Bilateral conjunctival injection (not associated w/exudates)
  • Injected or fissured lips, injected pharynx or strawberry tongue
  • Erythema of palms/soles, or edema of hands/feet (acute phase)
  • Periungual desquamation (convalescent phase)
  • Polymorphous rash
  • Maculopapular/scarlatiniform rash on trunk
  • Cervical adenopathy
  • Erythematous oral mucosa
  • Desquamation of hands and feet
  • Other findings in cardiovascular, respiratory, musculoskeletal, GI, GU and nervous systems
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Risk Factors for Kawasaki Disease

  • Japanese descent>African Americans/Filipinos>Caucasian
  • Age less than 5 years with peak incidence 18 to 24 months
  • Cases appear year round – most often spring or winter
  • Most commonly seen in US and Japan
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Differential Diagnosis

  • Adenovirus
  • Echovirus
  • Epstein-barr virus
  • Measles
  • Mercury hypersensitivity reaction
  • Rocky mountain spotted fever
  • Stevens-johnson syndrome
  • Toxic epidermal necrolysis
  • Toxic shock syndrome
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Diagnostic Testing

Text / literature information and recommendations include:
  1. Lab tests are not diagnostic but may be obtained to rule out other disorders.
    • CBC – may show anemia with a left shift in the acute phase. Children often have normocytic, normochromic anemia.
    • Platelets: thrombocytosis in 2nd week, up to 1,000,000/mm3.
    • LFTs, bilirubin – 30% of patients have mild to moderate elevation of transaminases. Some may develop jaundice from hydrops of the gallbladder.
    • UA – WBCs if clean catch. WBCs are of urethral origin and may be missed on catheterized specimens. Leukocyte esterase is negative.
    • ANA
    • RF
    • ESR - elevated
    • CRP - elevated
    • Pharyngeal and blood cultures
    • Cardiac Markers: CKMB and Troponin
    • Lumbar puncture – CSF can show elevated white cell counts.
    • Arthrocentesis of involved joints demonstrates 125,000 to 300,000 white cells/mm3.

  2. Imaging
    • CXR: evaluate for CHF and infiltrates
    • ECHO evaluate for aneurysms, pericardial fluid, valve disease
    • ECG: evaluate for arrhythmias
    • Cardiac Catheterization: evaluate for aneurysm
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Clinical Risk and Safety Pearls

Risk Factors for developing coronary artery aneurysms have been found:
  • In infants < 6 months of age, prolonged fever and irritability may be the only clinical manifestations.
  • Delayed diagnosis is common in older children and adolescents.
  • Fever and pyuria in an infant or young child can be mistakenly attributed to a UTI, and later development of rash, red eyes and red lips to an antibiotic reaction.
  • Diagnosis can be overlooked in an infant with prolonged fever/irritability and a culture-negative CSF pleocytosis suggestive of aseptic meningitis.
  • Cervical lymphadenitis may be misdiagnosed as bacterial adenitis.
  • Patients with shock may be misdiagnosed with bacterial sepsis or toxic shock syndrome.
  • Risk Factors associated with increased risk of coronary artery abnormalities include: late diagnosis and delayed treatment with IV Immune Globulin; age < 1 or> 9; male sex; fever ≥ 14 days; serum Na < 135 mEq/L; Hematocrit < 35%; WBC> 12,000mm3.
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Treatment

Common text / literature recommendations include:
  1. High-dose IV immune globulin (IVIG) as soon as possible, but within 10 days of illness onset
  2. Moderate- to high-dose ASA (aspirin)
  3. Hospital admission and consultation with pediatric cardiology
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Complications

  • 20% to 25% of patients develop cardiovascular sequelae (coronary artery aneurysms, myocardial infarction, myocarditis).
  • Infants less than one year of age with KD have the highest risk of cardiac complications.
  • Patients with fever for more than 16 days or with recurrence have a higher risk of developing an aneurysm.
  • Coronary artery aneurysms occur in 20% to 25% of untreated children while only 4% of adequately treated patients develop aneurysms
  • Non vascular complications
  • Acute renal failure
  • Abdominal: gallbladder hydrops, paralytic ileus, appendicular vasculitis
  • Behavioral changes

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  1. American Academy of Pediatrics. Kawasaki disease. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. American Academy of Pediatrics; 2018:490-497.
  2. Dietz SM, van Stijn D, Burgner D, et al. Dissecting Kawasaki disease: A state-of-the-art review. Eur J Pediatr. 2017;176(8):995-1009.
  3. Friedman KG, Gauvreau K, Hamaoka-Okamoto A, et al. Coronary artery aneurysms in Kawasaki disease: Risk factors for progressive disease and adverse cardiac events in the US population. J Am Heart Assoc. 2016;5(9).
  4. McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals from the American Heart Association. Circulation. 2017;135(17):e927-e999.
  5. Newburger JW. Kawasaki disease: State of the art. Congenit Heart Dis. 2017;12(5):633-635.
  6. Newburger JW, Takahashi M, Burns JC. Kawasaki disease. J Am Coll Cardiol. 2016;67(4):1738-1749.

This is intended solely as reference material and is not a recommendation for any specific patient. The practitioner must rely upon his or her own professional judgment and medical decision-making to determine whether it is relevant in a particular case. Materials are derived from medical and nursing texts, medical literature and national guidelines and should not be considered complete or authoritative. Users must rely on specific patient presentation, experience and judgment when utilizing any of the information contained herein relative to an actual patient.
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