Kawasaki’s disease was first reported in 1961 by Dr Tomisaku Kawasaki in a 4-year-old boy and he went on over the next to report nearly 50 such cases. This was recognised as an acute illness only after 1970 and its cardiac complications could be better studied only in 1976. Recently, Munawar Faruqui opened up about his 1.5-year-old son who was suffering from Kawasaki disease.
Symptoms of Kawasaki disease
When we spoke to Dr Soundarya M, Consultant Pediatrics, KMC Hospital, Mangalore, he said that Kawasaki’s disease is characterised by very indistinct clinical features. The classical symptoms include persistently high-grade fever (5 or more days), red, dry and fissured lips, a red tongue called ‘Strawberry tongue’, reddish eyes, and painful swelling of the lymph glands in the neck. Other associated features are irritability in smaller children, minimal swelling of hands and feet, and peeling of skin around the nails in the second to third week of the illness. In countries where BCG is given, the BCG site will also show a sudden reactivation with erythema and redness around the scar. When blood tests are performed, the reports show features that overlap with infection i,e., elevated inflammatory markers, however, unless this disease is appropriately identified and treated, the fever and other symptoms do not respond to routine antibiotic treatments. All the features of Kawasaki’s disease are not seen in all children and the presence of some of these features should raise suspicions for evaluation.
The importance of early diagnosis
The importance of early and prompt recognition of this condition is because of its complications. Kawasaki’s disease can cause the development of coronary aneurysms which are dilated areas on the blood vessels supplying the heart and can occur in up to 25% of children who have this disease. These aneurysms can rupture, get blocked with a clot or cause poor blood flow to the heart resulting in acute catastrophic cardiac events. Early recognition and treatment help prevent this complication from occurring.
There has been no specific cause identified for Kawasaki’s disease. As this disease is more frequent in certain Asian populations, a genetic cause has been identified as a variation in the ITPKC gene which controls the activity of the T immune cells. However, an infection could be the triggering event. This infection can cause activation of the immune system and the production of numerous inflammatory molecules, typically an IgA response. These activated immune molecules mount an inflammatory response towards the skin and mucous membranes, blood vessels supplying the heart, causing the clinical features and complications.
Treatment for Kawasaki disease
Kawasaki’s disease needs to be treated early with intravenous immunoglobulin therapy, preferably within the first 10 days of the illness (though the earlier the initiation, the complications are lesser). Along with this aspirin is also used till the inflammation reduces. Only in case of poor or incomplete response to this primary treatment line, alternative treatment options including steroids are considered. The important take-home message in Kawasaki’s disease is that all fevers are not infections and antibiotics are not the solution. Once the diagnosis of Kawasaki’s disease is made, prompt initiation of therapy will help in controlling the inflammation and avoiding cardiac complications. Regular follow-ups for these children to check their inflammatory markers and cardiac scans are needed to ensure a return to complete normalcy.
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