Japan has the highest incidence in the world, with an annual incidence of 300/100,000 children under the age of four years 10. Worldwide, it is the commonest vasculitis in children 9. It is slightly more common in males, M: F, 1.4:1 10.
It is more common in siblings, ~3% of siblings will be diagnosed with Kawasaki, this equates to a ten-fold increased risk in a sibling. It is also more likely in children of affected parents, although no genetic link has been identified 10.
In the United States, it is most commonly seen in children of Asian ethnicity, in particular, Japanese, but less commonly in African Americans and least commonly in Caucasians.
Since April 2020, rare cases of a Kawasaki-like disorder have been reported in critically-ill children who are COVID-19 positive. A true causative association between SARS-CoV-2, the cause of COVID-19, and Kawasaki disease has not been established as yet 14,15.
Persistent fever is the commonest way with which most children present. The specific signs and symptoms vary with the stage of the disease:
- fever that is refractory to antibiotics
- general malaise and irritability
- non-exudative conjunctivitis (90%)
- anterior uveitis (70%)
- perianal erythema (70%)
- lymphadenitis (75%)
- strawberry tongue
- desquamation of the digits
- coronary artery aneurysms
- highest risk of death
- clinical symptoms and signs recede
- persistence of any cardiac complications into adulthood
An autoimmune etiology has been postulated. It is generally self-limiting but acute fatalities are thought to occur in ~1% of cases. The most concerning morbidity is due to coronary involvement where it can manifest as myocarditis with coronary arterial aneurysm formation 1,2,4.
Rarely, a few years after resolution of the initial episode, the patient may present with calcified coronary artery aneurysms visible on the chest x-ray 8. Although rare, this is an Aunt Minnie presentation of Kawasaki disease sequelae in older patients.
Coronary angiography / CT angiography
Coronary artery aneurysms can be classified as 11:
- small: <5 mm
- medium: 5–8 mm
- large: >8 mm
Coronary stenosis is more common in the left coronary artery while intracoronary thrombus is more frequently observed in the right coronary artery 11.
MRI / MR angiography
Useful in assessing myocardial perfusion, wall thinning and aneurysms.
Transthoracic echocardiography especially useful in the approach to patients who fall short of full clinical criteria (incomplete KD), as the presence of the following is diagnostic 12:
coronary artery dilation
- dilation which exceeds the mean coronary artery diameters by 2.5 standard deviations is classified as aneurysmal
- left ventricular dysfunction
- manifestation of endocardial inflammation
- pericardial effusion
History and etymology
It is named after the Japanese pediatrician Tomisaku Kawasaki (1925-2020) 9,16 who saw his first case in 1961 and initially described it in a case series of 50 children in 1967 7,9.
- 1. Chung CJ, Stein L. Kawasaki disease: a review. Radiology. 1998;208 (1): 25-33. Radiology (citation) - Pubmed citation
- 2. Frey EE, Matherne GP, Mahoney LT et-al. Coronary artery aneurysms due to Kawasaki disease: diagnosis with ultrafast CT. Radiology. 1988;167 (3): 725-6. Radiology (abstract) - Pubmed citation
- 3. Bisset GS, Strife JL, Mccloskey J. MR imaging of coronary artery aneurysms in a child with Kawasaki disease. AJR Am J Roentgenol. 1989;152 (4): 805-7. AJR Am J Roentgenol (citation) - Pubmed citation
- 5. Duerinckx AJ, Troutman B, Allada V et-al. Coronary MR angiography in Kawasaki disease. AJR Am J Roentgenol. 1997;168 (1): 114-6. AJR Am J Roentgenol (citation) - Pubmed citation
- 6. Takemura A, Suzuki A, Inaba R et-al. Utility of coronary MR angiography in children with Kawasaki disease. AJR Am J Roentgenol. 2007;188 (6): W534-9. doi:10.2214/AJR.05.1414 - Pubmed citation
- 7. Kawasaki T. Acute Febrile Mucocutaneous Syndrome With Lymphoid Involvement With Specific Desquamation of the Fingers and Toes in Children. Jpn J Allergy 1967;16: 178-182
- 8. Momolli MK, Castro E Silva Pretto JL, Sato D et-al. Calcified aneurysms in coronary arteries of a 48-year-old patient. Arq. Bras. Cardiol. 2001;76 (3): 255-60. Pubmed citation
- 9. Kawasaki T, Singh S. Kawasaki disease - the journey over 50 years: 1967-2017. (2018) International journal of rheumatic diseases. 21 (1): 7-9. doi:10.1111/1756-185X.13215 - Pubmed
- 10. Nakamura Y. Kawasaki disease: epidemiology and the lessons from it. (2018) International journal of rheumatic diseases. 21 (1): 16-19. doi:10.1111/1756-185X.13211 - Pubmed
- 11. Broncano J, Vargas D, Bhalla S, Cummings KW, Raptis CA, Luna A. CT and MR Imaging of Cardiothoracic Vasculitis. (2018) Radiographics : a review publication of the Radiological Society of North America, Inc. 38 (4): 997-1021. doi:10.1148/rg.2018170136 - Pubmed
- 12. McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, Baker AL, Jackson MA, Takahashi M, Shah PB, Kobayashi T, Wu MH, Saji TT, Pahl E. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. (2017) Circulation. 135 (17): e927-e999. doi:10.1161/CIR.0000000000000484 - Pubmed
- 13. Jeudy J, White CS, Kligerman SJ, Killam JL, Burke AP, Sechrist JW, Shah AB, Hossain R, Frazier AA. Spectrum of Coronary Artery Aneurysms: From the Radiologic Pathology Archives. (2018) Radiographics : a review publication of the Radiological Society of North America, Inc. 38 (1): 11-36. doi:10.1148/rg.2018170175 - Pubmed
- 14.Jones VG, Mills M, Suarez D, Hogan CA, Yeh D, Bradley Segal J, Nguyen EL, Barsh GR, Maskatia S, Mathew R. COVID-19 and Kawasaki Disease: Novel Virus and Novel Case. (2020) Hospital pediatrics. doi:10.1542/hpeds.2020-0123 - Pubmed
- 15. Mahase E. Covid-19: concerns grow over inflammatory syndrome emerging in children. (2020) BMJ (Clinical research ed.). 369: m1710. doi:10.1136/bmj.m1710 - Pubmed
- 16. Kermode-Scott Barbara. Tomisaku Kawasaki: identified and named Kawasaki disease BMJ 2020; 370 :m3091 doi:10.1136/bmj.m3091