• Clinical science

Kawasaki disease (Mucocutaneous lymph node syndrome)

Summary

Kawasaki disease is an acute, necrotizing vasculitis of unknown etiology. The condition primarily affects children under the age of five and is more common among those of Asian descent. The disease is characterized by a high fever, desquamative rash, conjunctivitis, mucositis (e.g., “strawberry tongue”), cervical lymphadenopathy, as well as erythema and edema of the distal extremities. However, coronary artery aneurysms are the most concerning possible manifestation as they can lead to myocardial infarction or arrhythmias. Kawasaki disease is a clinical diagnosis, further supported by findings such as elevated ESR or evidence of cardiac involvement on echocardiography. Treatment with IV immunoglobulins and high-dose aspirin is essential and should be initiated immediately after diagnosis.

Epidemiology

  • Sex: > (1.5:1) [1]
  • Age: primarily children < 5 years (most common cause of acquired coronary artery disease in children) [2][3]
  • Peak incidence: occurs mostly in late winter and spring
  • Prevalence [2]
    • Approx. 20 per 100,000 children
    • Highest rate in children of Asian and Pacific-Islander descent
  • Mortality: < 0.5% [4]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

  • The exact cause of Kawasaki disease remains uncertain.
  • However, it is associated with infectious and genetic factors (the prevalence is higher in patients of Asian descent and in siblings of affected children). [5]

Clinical features

  • Clinical diagnosis requires fever for at least 5 days and one of the following: [6]
    • ≥ 4 other specific symptoms
    • < 4 specific symptoms and involvement of the coronary arteries
  • Specific symptoms include:
    • Erythema and edema of hands and feet, including the palms and soles (the first week)
    • Possible desquamation of fingertips and toes after 2–3 weeks
    • Polymorphous rash, originating on the trunk
    • Painless bilateral “injected” conjunctivitis without exudate
    • Oropharyngeal mucositis
    • Cervical lymphadenopathy (mostly unilateral)
  • Nonspecific symptoms may precede the onset of Kawasaki disease (e.g., diarrhea, fatigue, abdominal pain)

Always consider Kawasaki disease in small children with a rash and high fever unresponsive to antibiotics.A SHARK is riding a Fast Kawasaki: Strawberry tongue, Hands and feet may show desquamation, Adenopathy, Rash, K(c)onjunctivitis, and Fever for ≥ 5 days. are the most common features of Kawasaki disease.

Diagnostics

Kawasaki disease is a clinical diagnosis (see “Clinical features” above) which is supported by the following diagnostic findings:

Treatment

  • IV immunoglobulin (IVIG)
    • High single-dose to reduce the risk of coronary artery aneurysms
    • Most effective if given within the first 10 days following disease onset [8]
  • High-dose oral aspirin [9][10]
  • IV glucocorticoids: may be considered in addition to standard treatment, esp. in cases of treatment-refractory disease, as they lower the risk of coronary involvement

To avoid the risk of Reye syndrome, children should not be treated with aspirin, especially if a viral infection is suspected. Kawasaki disease is an exception to this rule.

Complications

We list the most important complications. The selection is not exhaustive.

  • 1. Huang WC, Huang LM, Chang IS, Chang LY, Chiang BL, Chen PJ, Wu MH, Lue HC, Lee CY, Kawasaki Disease Research Group. Epidemiologic features of Kawasaki disease in Taiwan, 2003-2006. Pediatrics. 2009; 123(3): pp. e401–5. doi: 10.1542/peds.2008-2187.
  • 2. Holman RC, Belay ED, Christensen KY, Folkema AM, Steiner CA, Schonberger LB. Hospitalizations for Kawasaki syndrome among children in the United States, 1997-2007. The Pediatric infectious disease journal. 2010; 29(6): pp. 483–8. doi: 10.1097/INF.0b013e3181cf8705.
  • 3. Saguil A, Fargo M, Grogan S. Diagnosis and management of kawasaki disease. American family physician. 2015; 91(6): pp. 365–71. pmid: 25822554.
  • 4. de La Harpe M, di Bernardo S, Hofer M, Sekarski N. Thirty Years of Kawasaki Disease: A Single-Center Study at the University Hospital of Lausanne. Frontiers in pediatrics. 2019; 7: p. 11. doi: 10.3389/fped.2019.00011.
  • 5. Uehara R, Yashiro M, Nakamura Y, Yanagawa H. Kawasaki disease in parents and children. Acta paediatrica (Oslo, Norway : 1992). 2003; 92(6): pp. 694–7. doi: 10.1080/08035320310002768.
  • 6. AHA. Diagnostic Guidelines for Kawasaki Disease. Circulation. 2001; 103(2): pp. 335–336. doi: 10.1161/01.CIR.103.2.335.
  • 7. Newburger JW, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation. 2004; 110(17): pp. 2747–71. doi: 10.1161/01.CIR.0000145143.19711.78.
  • 8. Oates-Whitehead RM, Baumer JH, Haines L, Love S, Maconochie IK, Gupta A, Roman K, Dua JS, Flynn I. Intravenous immunoglobulin for the treatment of Kawasaki disease in children. The Cochrane database of systematic reviews. 2003: p. CD004000. doi: 10.1002/14651858.CD004000.
  • 9. Newburger JW, Takahashi M, Beiser AS, Burns JC, Bastian J, Chung KJ, Colan SD, Duffy CE, Fulton DR, Glode MP. A single intravenous infusion of gamma globulin as compared with four infusions in the treatment of acute Kawasaki syndrome. The New England journal of medicine. 1991; 324(23): pp. 1633–9. doi: 10.1056/NEJM199106063242305.
  • 10. Newburger JW, Takahashi M, Burns JC, Beiser AS, Chung KJ, Duffy CE, Glode MP, Mason WH, Reddy V, Sanders SP. The treatment of Kawasaki syndrome with intravenous gamma globulin. The New England journal of medicine. 1986; 315(6): pp. 341–7. doi: 10.1056/NEJM198608073150601.
  • 11. Kato H, Sugimura T, Akagi T, Sato N, Hashino K, Maeno Y, Kazue T, Eto G, Yamakawa R. Long-term consequences of Kawasaki disease. A 10- to 21-year follow-up study of 594 patients. Circulation. 1996; 94(6): pp. 1379–85. doi: 10.1161/01.cir.94.6.1379.
last updated 10/15/2020
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