A 3-year-old Japanese boy presents to the emergency department with a 6-day fever and an erythematous rash with edema on both extremities. His mother states that her child has not been well for 6 days and that the fever does not subside with acetaminophen use. Physical examination reveals bilateral conjunctivitis, a red bright tongue and bilateral cervical adenopathy. There is no evidence of tonsillitis or peritonsillar abscess. The mother denies having cough, runny nose, or difficulty breathing the last 5 days. Laboratory work up reveals a white blood cell (WBC) count of 14,000/µl, Hb of 10 gr/dl and a platelet count of 130,000/µl. The patient undergoes an echocardiogram which detects a 5 mm dilatation of the left descending coronary artery (LAD). Treatment with intravenous immunoglobulin and aspirin is immediately initiated. After 5 weeks of treatment with low dose aspirin the LAD dilation resolves completely.
Which of the following would be part of the patient's long term-follow up?
The correct answer is: D. Cardiovascular risk factor assessment after 1 year
This 3-year old patient presents with persistent high fever acccompanied by red "strawberry tongue", bilateral conjuctivitis and cervival adenopathy, as well as an erythematous rash in both extremities. This clinical presentation represents classic Kawasaki disease. It is an acute, self-limiting medium-vessel vasculitis, with a predilection for coronary arteries. It affects primarily male children younger than 5 years old.1,2
Although the etiology is not well understood, environmental and genetic factors have been recently proposed for the pathogenesis of the disease. Criteria for the diagnosis of classic Kawasaki disease are:
Fever (>38.5°C) and at least 4 out of 5 following principal clinical features:
Bilateral conjuctivitis without exudate
Maculopapular or erythematous rash
Cervical adenopathy (≥1.5 cm diameter)
Erythema and cracking of lips, oral mucosa, and/or strawberry tongue
Erythema and edema of the hands and feet in acute phase with subsequent desquamation1,2
An echocardiogram should be undertaken in the acute phase of illness because the major complication of the disease and primary cause of mortality is the formation of coronary artery aneurysms. Treatment should be initiated quickly with high dose intravenous immunoglobulin and low dose aspirin until the resolution of fever.1
In this patient echocardiography reveals a 5-mm dilation of LAD that is resolved after 5 weeks treatment with low dose aspirin. According to the American College of Cardiology/American Heart Association (ACC/AHA) guidelines, patients with an initial presentation of Kawasaki disease accompanied by a coronary artery dilation that is completely resolved after standard treatment should have a cardiovascular risk factor assessment at least 1 year after the episode of acute Kawasaki disease.1 (Answer D)
Low dose aspirin for 1 year (Answer A) is indicated until the complete resolution of coronary artery dilation. These dilations most commonly regress after 1 year of aspirin therapy, with no further cardiologic assessment being required. This patient has a complete resolution of the coronary artery dilation after 5 months of treatment, that requires no further treatment with aspirin.1
B-blockers and anticoagulation prophylaxis for 1 year (Answer B), as well as cardiologic assessment every 3 months for 1 year (Answer C) after the initial episode of Kawasaki disease, are indicated for large and giant aneurysms that do not resolve.1
Kawasaki disease is a medium- and small-vessel vasculitis with higher prevalence among children younger than 5 years old. The major complication of the disease is coronary artery aneurysm formation and cardiac death in young patients. Coronary artery dilatation usually regresses in one year, requiring no further medical treatment and cardiologic assessment. However, cardiovascular risk factor and physical activity counseling at 1 year after the initial episode of Kawasaki disease is recommended.1,2
McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation 2017;135:e927-e999.
Saguil A, Fargo M, Grogan S. Diagnosis, and management of kawasaki disease. Am Fam Physician 2015;91:365-71.