top of page

Understanding and detecting Kawasaki disease on time

Last updated:

06/01/25, 12:26

Published:

06/02/25, 08:00

A rare disease that causes inflammation in the blood vessels

What is Kawasaki disease?


Kawasaki disease is a rare type of vasculitis that damages blood vessels through inflammation and is prevalent in children under the age of five. Kawasaki disease is predominantly found in children of Asian races–mainly in Japan, Korea, Taiwan, and Asian races in the US–and is the leading cause of acquired heart disease in children in most developed countries. 


What causes Kawasaki disease?


There is no known cause of Kawasaki disease, however, studies suggest a link between genetics and the disease, noting a high incidence between siblings and in children with a parental history of Kawasaki disease. Another study provided further evidence of genetic susceptibility, stating that variation in the expression of CASP3 and ITPKC—genes heavily involved in T cell function—leads to an overexpression of T cells.This can be attributed to the inflammatory symptoms of the disease. 


There are speculations that it may be caused by an airborne agent originating in Central Asia which moves across different geographical regions. This study suggests that through winds, the airborne agent is able to cause Kawasaki disease via infection of the respiratory tract–further investigation is needed regarding this hypothesis. 


Diagnosing Kawasaki disease


Symptoms of Kawasaki disease, which are often accompanied by a fever, are classified into three phases: acute, subacute, and convalescent. 


The acute phase usually lasts between two to three weeks and symptoms include:

  • Carditis 

  • Mucosal inflammation (cracked and dry lips, strawberry tongue, swollen lymph nodes)

  • Polymorphous rash 

  • Coronary artery aneurysms 


The subacute phase also lasts up to three weeks and includes symptoms such as:

- Perineal and periungual desquamation 

- Arthralgia 

- Myocardial disease 


The convalescent phase is when most clinical signs dissolve and usually lasts up to three months. It is important to note that while most symptoms clear up during this phase, cardiac issues may still persist in some patients. 


Misdiagnosing Kawasaki disease is very common as its symptoms are similar to that of many diseases like scarlet fever or toxic shock syndrome. With that being said, confirming its diagnosis is often a case of ruling out these diseases. In addition to identifying symptoms linked to other diseases, conducting laboratory tests such as CRP, CBC, and ESR can help confirm a diagnosis of Kawasaki disease. Additionally, echocardiograms and electrocardiograms can help assess coronary abnormalities as well as overall heart function. 


Treating Kawasaki disease


Following diagnoses, patients are first administered an IVIG and a high dose of aspirin to reduce inflammation as well as eliminate pain, swelling and fever. Patients are then administered lower doses of aspirin which helps prevent blood clotting. 


Roughly 25% of untreated patients are at a higher risk of developing coronary artery aneurysms and lasting cardiovascular issues in general. This risk drops down to 5% when treated appropriately. IVIG is proven to be effective in treating approximately 85-90% of cases when administered within the first ten days of the illness which is why it is imperative that patients are treated early. 


X-rays are regularly conducted on patients as they can help visualise blood vessels and potential heart abnormalities that may suggest further complications. It can also observe the effectiveness of treatment over time. Post-recovery, an echocardiogram is recommended periodically to detect any coronary abnormalities that may have developed much later on. 


Summary


Kawasaki disease is a rare disease that causes inflammation in the blood vessels. It normally develops in children under the age of five and is yet to have a known cause. It is often hard to diagnose as its symptoms are similar to that of other diseases, which is why it is important to identify its symptoms (polymorphous rash, mucosal inflammation, desquamation, etc) as well as conduct tests such as CBC, CRP, ESR, an electrocardiogram, etc to help rule out other diseases. It is essential that children with Kawasaki disease are diagnosed and treated early as this can help treat coronary artery aneurysm and prevent lasting coronary and cardiovascular abnormalities. 


Written by Sherine Latheef


Related articles: Sideroblastic anaemia / Blood / Inflammation therapy



REFERENCES


Onouchi, Y., Ozaki, K., Buns, J.C., Shimizu, C., Hamada, H., Honda, T., Terai, M., Honda, A., Takeuchi, T., Shibuta, S., Suenaga, T., Suzuki, H., Higashi, K., Yasukawa, K., Suzuki, Y., Sasago, K., Kemmotsu, Y., Takatsuki, S., Saji, T. and Yoshikawa, T. (2010). Common variants in CASP3 confer susceptibility to Kawasaki disease. Human Molecular Genetics, 19(14), pp.2898–2906. doi:https://doi.org/10.1093/hmg/ddq176. 


Agarwal, S. and Agrawal, D.K. (2017). Kawasaki Disease: Etiopathogenesis and Novel Treatment Strategies. Expert review of clinical immunology, [online] 13(3), pp.247–258. doi:https://doi.org/10.1080/1744666X.2017.1232165. 


Wolff, A.E., Hansen, K.E. and Zakowski, L. (2007). Acute Kawasaki Disease: Not Just for Kids. Journal of General Internal Medicine, [online] 22(5), pp.681–684. doi:https://doi.org/10.1007/s11606-006-0100-5. 


Oh, J.-H., Cho, S. and Choi, J.A. (2023). Clinical Signs of Kawasaki Disease from the Perspective of Epithelial-to-Mesenchymal Transition Recruiting Erythrocytes: A Literature Review. Reviews in Cardiovascular Medicine, 24(4), pp.109–109. doi:https://doi.org/10.31083/j.rcm2404109. 


Team, H.J. (2018). Kawasaki Disease - Causes, Signs, Symptoms,Treatment. [online] Health Jade. Available at: https://healthjade.com/kawasaki-disease/.

Project Gallery

bottom of page