Kawasaki disease | |
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Other names | Kawasaki syndrome,[1] mucocutaneous lymph node syndrome[2] |
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A medical illustration depicting Kawasaki disease. | |
Specialty | Pediatrics, Rheumatology, Immunology |
Symptoms | Fever > 5 days, large lymph nodes, rash, sore throat, diarrhea[1] |
Complications | Coronary artery aneurysms[1] |
Usual onset | < 5 years old[1] |
Duration | ~ 3 weeks[1] |
Causes | Unknown[1] |
Risk factors | Age of < 5 years old |
Diagnostic method | Based on symptoms, ultrasound of the heart[1] |
Differential diagnosis | Scarlet fever, juvenile rheumatoid arthritis, paediatric multisystem inflammatory syndrome[3][1] |
Medication | Oral Aspirin and intravenous immunoglobulin[1] |
Prognosis | Mortality 0.2% with treatment[4] |
Frequency | 8–124 per 100,000 people under five[5] |
Named after | Tomisaku Kawasaki |
Kawasaki disease (also known as mucocutaneous lymph node syndrome) is a syndrome of unknown cause that results in a fever and mainly affects children under 5 years of age.[6] It is a form of vasculitis, where medium-sized blood vessels become inflamed throughout the body.[1] The fever typically lasts for more than five days and is not affected by usual medications.[1] Other common symptoms include large lymph nodes in the neck, a rash in the genital area, lips, palms, or soles of the feet, and red eyes.[1] Within three weeks of the onset, the skin from the hands and feet may peel, after which recovery typically occurs.[1] The disease is the leading cause of acquired heart disease in children in developed countries, which include the formation of coronary artery aneurysms and myocarditis.[1][7]
While the specific cause is unknown, it is thought to result from an excessive immune response to particular infections in children who are genetically predisposed to those infections.[6] It is not an infectious disease, that is, it does not spread between people.[8] Diagnosis is usually based on a person's signs and symptoms.[1] Other tests such as an ultrasound of the heart and blood tests may support the diagnosis.[1] Diagnosis must take into account many other conditions that may present similar features, including scarlet fever and juvenile rheumatoid arthritis.[9] An emerging "Kawasaki-like" disease temporally associated with COVID-19[10] appears to be a distinct syndrome.[11]
Typically, initial treatment of Kawasaki disease consists of high doses of aspirin and immunoglobulin.[1] Usually, with treatment, fever resolves within 24 hours and full recovery occurs.[1] If the coronary arteries are involved, ongoing treatment or surgery may occasionally be required.[1] Without treatment, coronary artery aneurysms occur in up to 25% and about 1% die.[4][12] With treatment, the risk of death is reduced to 0.17%.[12] People who have had coronary artery aneurysms after Kawasaki disease require lifelong cardiological monitoring by specialized teams.[13]
Kawasaki disease is rare.[1] It affects between 8 and 67 per 100,000 people under the age of five except in Japan, where it affects 124 per 100,000.[5] Boys are more commonly affected than girls.[1] The disorder is named after Japanese pediatrician Tomisaku Kawasaki, who first described it in 1967.[5][14]
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