Kawasaki’s DiseaseDz

Synonym: Mucocutaneous lymph node syndrome.

ICD-9 Code: 446.1.

Definition: Kawasaki’s disease is an acute febrile illness of children.

Etiology: Although a variety of infectious organisms and other environmental agents have been proposed to be of etiologic relevance, none has been conclusively implicated. However, there is evidence of restricted clonality of T cells in patients with Kawasaki’s disease, strongly suggesting an immune re- sponse to an as yet unidentified antigen.

Demographics: Most affected patients are under 5 years old, with many cases arising in infancy. There is a slight (~1.4:1) female preponderance. It is most common in Japan but is increasingly seen in China, the United States, and England. It is considered an uncommon disease, with an incidence of four to 15 cases per 100,000 younger than the age of 5 years.

Cardinal Findings: Typically, Kawasaki’s disease presents acutely. After a few days of intermittent or consistent fever, patients develop conjunctival injection, swelling of the lips and oral mucous membranes, cervical lymphadenopathy, and rash. An erythematous macular rash may appear on the trunk, limbs, hands, or feet and may be accompanied by diffuse swelling. Skin lesions may resolve with desquamation. Coronary aneurysms may occur early in the first few weeks of disease. The prevalence of these lesions decreases from 20% to 10% to 5% at 1 month, 2 months, and 1 year, respectively. Aneurysms >8 mm in diameter are less likely to regress. Potential sequelae of coronary arterial involvement with Kawasaki disease include rupture and myocardial infarction. The mortality rate is ~0.1%.

Diagnostic Testing: Common abnormalities include leukocytosis, thrombocytosis, elevated transaminases, CRP, and ESR. Electrocardiogram abnormalities are common.

Diagnostic Criteria: Diagnostic features include: (a) fever for >5 days; (b) erythema of the palms or soles with edema or desquamation in the convalescent stage; (c) polymorphous truncal rash; (d) bilateral conjunctival injection; (e) erythema of the lips, strawberry tongue, or oral mucosa injection; and (f) acute nonpurulent cervical lymphadenopathy. A diagnosis of Kawasaki’s disease requires that five of the six criteria be fulfilled or that four criteria be present with evidence of coronary aneurysm by echocardiography or angiography.

Therapy: Treatment is directed primarily at preventing development or progression of coronary aneurysms. The current standard therapy consists of high-dose intravenous gamma-globulin (2 g/kg IgG, administered as either a single dose or in split doses, e.g., 400 mg/kg for 5 days) in conjunction with aspirin (30–100 mg/kg/day). Aspirin may be continued in patients with persistent aneurysms until the lesions resolve.

BIBLIOGRAPHY

Burns JC. Kawasaki disease. Adv Pediatr 2001;48:157–177.PMID:11480756

Kato H, Ichinose E, Kawasaki T. Myocardial infarction in Kawasaki disease: clinical analysis in 195 cases. J Pediatr 1986;108:923–927.PMID:3712157