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Kawasaki Syndrome

Kawasaki Syndrome is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • Kawasaki syndrome (KS) is a self-limited acute, febrile, systemic vasculitis of small- and medium-sized arteries that predominantly affects patients age 6 months to 5 years and is the most prominent cause of acquired coronary artery disease in pediatric populations.
    • Vasculitis of coronary arteries resulting in aneurysms/ectasia, further leading to myocardial infarction (MI)/ischemia or sudden death
  • System(s) affected: cardiovascular, gastrointestinal, hematologic/lymphatic/immunologic, musculoskeletal, nervous, pulmonary, renal/urologic, skin/exocrine
  • Synonym(s): mucocutaneous lymph node syndrome (MCLS), infantile polyarteritis, Kawasaki disease

ALERT
KS should be considered in any child with extended high fever unresponsive to antibiotics or antipyretics, rash, and nonexudative conjunctivitis.

Epidemiology

Incidence
  • Worldwide: affects all races but most prevalent in Asia; Japan annual incidence rate 265/100,000 in children <5 years of age
  • In the United States, the annual incidence in children <5 years is 19/100,000. In comparison to Caucasians, African Americans have a 1.5 times risk, and Asian Americans have a 2.5 times increased risk. Highest state incidence is in Hawaii.
  • Leading cause of acquired heart disease in children in developed countries
    • Predominant age: 1 to 5 years
    • 85% of cases are children <5 years of age and 50% <2 years of age.
    • Male-to-female ratio = 1.5:1

Prevalence
  • Highest to lowest prevalence: Asians > African Americans > Hispanics > Caucasians
  • Seasonal variation: increased in winter and early spring in temperate places, summer in Asia, and outbreaks at 2- to 3-year intervals

Etiology and Pathophysiology

  • Acute KS causes a necrotizing arteritis in the smooth muscle layer of medium extraparenchymal arteries that destroys arterial walls into the adventitia, especially in coronary arteries.
  • Inflammatory cells in the media secrete cytokines (TNF-α), interleukins 1 and 6, and matrix metalloproteases that cause fragmentation of the internal elastic lamina.
  • A prominence of IgA plasma cells and IgA deposits are characteristic features and may be found in the lungs.
  • As the acute process resolves, active neutrophilic inflammatory cells are succeeded by a subacute/chronic, lymphocytic vasculitis; fibroblasts and monocytes cause tissue repair/remodeling that may cause vascular fibrosis and stenosis.
  • Unknown; believed to be an exaggerated immune response to infectious agent due to the acute, self-limited nature; community-wide outbreaks; age distribution; seasonality; and laboratory features indicating respiratory route of entry

Genetics
  • Siblings of patients in Japan have a 10- to 30-fold increased risk, and >50% develop KS within 10 days of first case; increased occurrence of KS in children whose parents also had illness in childhood
  • Populations at higher risk and family link suggest a genetic predisposition.
  • Single-nucleotide polymorphisms in six different genes have been implicated in KS (Fcγ receptor 2A, CASP3, HLA class II, B-cell lymphoid kinase, IPTKC, CD40).
  • Coronary aneurisms are associated with variants in TGF-β signaling pathways.

General Prevention

No preventive measures available

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Citation

Stephens, Mark B., et al., editors. "Kawasaki Syndrome." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116332/all/Kawasaki_Syndrome.
Kawasaki Syndrome. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116332/all/Kawasaki_Syndrome. Accessed April 24, 2019.
Kawasaki Syndrome. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116332/all/Kawasaki_Syndrome
Kawasaki Syndrome [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 April 24]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116332/all/Kawasaki_Syndrome.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Kawasaki Syndrome ID - 116332 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116332/all/Kawasaki_Syndrome PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -