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Kawasaki Disease – An unrecognised killer
The fact that the disease most commonly affects children under the age of five makes it extremely important to consider Kawasaki disease a possibility in any febrile illness in this age group. What causes the illness? The mechanism or agent causing the illness remains unknown and is the subject of a lot of on-going investigation. How to recognise the Kawasaki disease? The principal clinical findings are: At least five of the above six features are required to make the diagnosis.
The illness typically starts with high persistent fever usually lasting for at least five days to sometimes 3-4 weeks. This is followed, in 2-3 days, by redness of both the eyes, red and sore lips and inside of the mouth, and tongue with discrete red and white spots (strawberry appearance). The rash appears as patches on the skin, in 3-5 days, following the initial onset of fever. Around the same time, firm swelling and redness of the hands and feet are seen. All these changes resolve spontaneously within a week. The most distinguishing feature of the illness, peeling of skin over the palms and soles, is seen, approximately, one to three weeks after the onset of the illness. The skin peeling tends to start in the palms first followed by the soles. The most important complication, the coronary artery changes, usually happen around day 10 of the illness. Nearly 95 % of these coronary aneurysms resolve on their own within a year, and until such time routine outpatient monitoring and aspirin therapy is essential. If the coronary changes persist beyond this period, longer follow-up is required. Treatment: The treatment advised is immunoglobulin infusion and high dose aspirin therapy, in the acute phase, until the fever subsides and blood results (white cell count, platelet count, and C-reactive Protein values) return to normal level. This is followed by low dose aspirin therapy, until echocardiography (heart ultrasound scan) rules out coronary changes or show that the aneurysms have resolved. Coronary angiography (shooting contrast dye into the coronary arteries) may be required to look for aneurysms in the coronary arteries if they are too difficult to be seen by the heart scan. A paediatric cardiologist must ideally manage the drug regime, and arrange serial follow-up of the patient with echocardiography until the aneurysm resolve. Learning points from this article: Any child under the age of 10, presenting itself with at least five days of high fever followed within 2-4 days by rash, redness in eyes, lips and oral cavity, followed later on by peeling of skin over palms and sole, should immediately be taken to a paediatric cardiologist to exclude Kawasaki disease. Such practice is strictly adhered to in the United Kingdom and may be more difficult in a tropical setting like India where numerous other illnesses may mimic Kawasaki disease initially, but the dangerous complication associated with this illness warrants early diagnosis and, therefore, early referral. Author profile: Dr. R. Prem Sekar, M.B.B.S., MRCP (Paediatrics)
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Kawasaki disease, previously known as mucocutaneous lymph node syndrome is a very much under-diagnosed illness and had recently gained notoriety in the U.K in view of one possibly fatal complication associated with it. The disease process may cause inflammation in the wall of the blood vessels, particularly the coronary arteries, which supply blood to the muscle of the heart. This may result in bulges (aneurysms) in the wall of the coronary arteries, thus predisposing to clot formation within the aneurysms. This has severe consequences as the blood supply to the muscle of the heart is compromised resulting in myocardial infarction (heart attack). With prompt diagnosis and appropriate treatment, it is reassuring, that only 5-10 % of patients develop coronary abnormalities.
Typical course of this illness:
