Kawasaki disease

Kawasaki disease (KD), also known as Kawasaki syndrome or mucocutaneous lymph node syndrome, is an acute systemic vasculitis that predominantly affects medium-sized arteries throughout the body, with a particular predilection for the coronary arteries. It primarily occurs in children under the age of 5, though it can occasionally affect older children and rarely adults. The disease was first described by Dr. Tomisaku Kawasaki in 1967 in Japan, where it remains most prevalent, particularly among children of East Asian descent. The hallmark clinical features of Kawasaki disease include prolonged high fever lasting at least five days, bilateral nonexudative conjunctival injection (red eyes without discharge), changes of the lips and oral mucosa (strawberry tongue, cracked lips), polymorphous skin rash, changes in the extremities (erythema and edema of hands and feet, followed by periungual desquamation), and cervical lymphadenopathy (typically unilateral). The most serious complication is coronary artery involvement, including coronary artery aneurysms, which can lead to myocardial infarction, sudden death, or long-term ischemic heart disease. Other cardiovascular manifestations may include myocarditis, pericarditis, and valvular dysfunction. The disease can also affect the gastrointestinal, musculoskeletal, and central nervous systems. The standard treatment for Kawasaki disease is high-dose intravenous immunoglobulin (IVIG) administered within the first 10 days of illness onset, combined with high-dose aspirin therapy. This treatment regimen has significantly reduced the incidence of coronary artery aneurysms from approximately 25% to less than 5%. Patients who do not respond to initial IVIG therapy (IVIG-resistant KD) may require additional IVIG doses, corticosteroids, infliximab, or other immunomodulatory agents. Long-term follow-up with echocardiographic monitoring is essential, particularly for patients who develop coronary artery abnormalities. The etiology remains unknown, though it is thought to result from an abnormal immune response to an infectious trigger in genetically susceptible individuals.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Kawasaki disease