Kawasaki Disease

Kawasaki disease is an acute febrile vasculitic syndrome of early childhood. While at the Tokyo Red Cross Medical Center in Japan, Tomisaku Kawasaki reported 50 children in 1961-1967 who presented with fever, rash, conjunctival injection, cervical lymphadenitis, inflammation of the lips and oral cavity, and erythema and edema of the hands and feet. Fatalities occurred in children younger than 2 years when they were improving or after they had seemingly recovered. Postmortem examinations revealed complete thrombotic occlusion of coronary-artery aneurysms (CAAs) with a myocardial infarction (MI) as the immediate cause of death.

Kawasaki disease is the leading cause of acquired heart disease in children in the developed world and may be a risk factor for adult ischemic heart disease.

The etiology of Kawasaki disease is unknown. Increasing evidence supports an infectious etiology for Kawasaki disease; however, whether the inflammatory response results from a conventional antigen or a superantigen continues to be debated.

Kawasaki disease has 3 stages, as follows:

Acute stage (1-11 d)

High fever (temperature >104°F)
Irritability
Nonexudative bilateral conjunctivitis (90%)
Anterior uveitis (70%)
Perianal erythema (70%)
Acral erythema and edema that impede ambulation
Strawberry tongue and lip fissures
Hepatic, renal, and GI dysfunction
Myocarditis and pericarditis
Lymphadenopathy (75%), generally a single, enlarged, nonsuppurative cervical node measuring approximately 1.5 cm

Subacute stage (11-30 d)

Persistent irritability, anorexia, and conjunctival injection
Decreased temperature
Thrombocytosis
Acral desquamation
Aneurysm forms
Convalescent or chronic phase (>30 d)
Expansion of aneurysm
Possible MI
A tendency for smaller aneurysms to resolve on their own (60% of cases)

Physical:

Patients with classic Kawasaki disease must have 5 of the following symptoms, with fever an absolute criterion:

1. Fever, lasting more than 5 days and refractory to appropriate antibiotic therapy
2. Polymorphous erythematous rash
3. Nonpurulent bilateral conjunctival injection
4. Oropharyngeal changes, including diffuse hyperemia, strawberry tongue, and lip changes (eg, swelling, fissuring, erythema, bleeding)
5. Peripheral extremity changes, including erythema, edema, induration, and desquamation
6. Nonpurulent cervical lymphadenopathy
Other findings may include the following:

General - Irritability
Cardiac - Coronary aneurysms, pericardial effusion, myocarditis, CHF
Neurologic - Stiff neck secondary to aseptic meningitis, facial palsy, cerebral infarction
Renal - Sterile pyuria, proteinuria, nephritis, acute renal failure
Musculoskeletal - Joint involvement (arthralgias or arthritis)
Pulmonary - Pleural effusion, infiltrates
GI - Abdominal pain, diarrhea, hepatitis, obstructive jaundice, hydrops, pancreatitis, gall bladder distention
Tissues - Meatitis, vulvitis, urethritis
Ophthalmologic - Conjunctivitis, uveitis
Dermatologic - Peripheral extremity gangrene, pustules, erythema multiforme–like lesions, perianal desquamation, macules, papules, measleslike rash, scarlet fever–like erythema, and induration at the site of bacille Calmette-Guérin (BCG) inoculation (commonly observed in Japan); pustulovesicular skin eruption in a child with probable Kawasaki disease; Beau lines associated with Kawasaki syndrome after the disease resolves

Up to 10-45% of published cases have incomplete or atypical clinical presentations. The 2 most commonly missing findings are cervical lymphadenopathy and polymorphous rash.

Mucous-membrane changes are the most common manifestations of Kawasaki disease, occurring in more than 90% of patients with either typical or atypical forms of the disease.

No specific laboratory test exists; however, certain abnormalities coincide with various stages.

Treatment:

The main goal of treatment is to prevent coronary artery disease and relieve symptoms. Full doses of salicylates (aspirin) and intravenous gammaglobulin are the mainstays of treatment.

1. Admit all patients to the hospital for intravenous gammaglobulin and observation until fever is controlled.
2. Closely monitor cardiovascular function.
3. Patients with small aneurysms must take aspirin.
4. Dipyridamole is indicated in patients with larger aneurysms.
5. Patients taking long-term aspirin therapy should receive an influenza vaccination to protect against Reye syndrome.