Kawasaki Disease

KEY FEATURES

ESSENTIALS OF DIAGNOSIS

• Fever lasting 5 or more days with at least 4 of the following:

  • – Bilateral nonexudative conjunctival injection

  • – Injected lips or pharynx, or “strawberry tongue”

  • – Acute nonsuppurative cervical lymphadenopathy

  • – Erythema of the palms and soles, or edema of the hands and feet

  • – Polymorphous exanthem

• Exclusion of common bacterial and viral infections

• Coronary artery aneurysms, myocarditis, and valve regurgitation

GENERAL CONSIDERATIONS

• May have a genetic and ethnic predisposition

• Also called mucocutaneous lymph node syndrome

• Acute vasculitis of unknown etiology, which occurs mostly in infants and children

• One of the most common vasculitides of childhood

• Heart failure may rarely complicate the acute phase of illness due to myocarditis

• Routine treatment with intravenous immune globulin (IVIG) and aspirin usually results in rapid clinical improvement

• Myocardial dysfunction during or after the second week of illness may suggest coronary artery aneurysm and resultant ischemia or infarction

• In severe cases, coronary or peripheral artery occlusions may occur and children may experience myocardial infarction, arrhythmias, or sudden death

• Kawasaki disease accounts for 5% of acute coronary syndromes in adults < 40 years of age

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

• Unexplained fever in an infant or child

• Skin rash

• Heart failure symptoms in patients with myocarditis in the first week of illness

• Chest pain, arrhythmias, or sudden death in patients

PHYSICAL EXAM FINDINGS

• Bilateral conjunctival injection

• Oral mucous membrane changes, including injected or fissured lips, injected pharynx, or “strawberry tongue”

• Palmar or solar erythema or edema

• Periungual desquamation

• Polymorphous rash

• Cervical lymphadenopathy

• Tachycardia out of proportion to the degree of fever

• Cardiac gallop sounds and muffled heart tones

• In severe cases, palpable brachial artery aneurysms; cold, pale, cyanotic, or rarely gangrenous digits

DIFFERENTIAL DIAGNOSIS

• Streptococcal and staphylococcal infections

• Measles, enterovirus, and adenovirus infections

• Systemic allergic reactions to medications

DIAGNOSTIC EVALUATION

LABORATORY TESTS

• Acute-phase reactants (C-reactive protein, erythrocyte sedimentation rate, and alpha₁-antitrypsin) elevation

• White blood count: leukocytosis and left shift in white blood count

• Reactive thrombocytosis

• Normocytic, normochromic anemia

• Pyuria of urethral origin (may be missed by bladder tap or catheterization)

• Elevated liver transaminase levels or hyperbilirubinemia due to intrahepatic congestion

• Mononuclear pleocytosis without low glucose or elevated protein in cerebrospinal fluid

ELECTROCARDIOGRAPHY

• Low R-wave voltages

• T-wave flattening

• Findings of acute myocardial infarction in rare cases

IMAGING STUDIES

• Echocardiography: small pericardial effusion, mild left ventricular dilatation, reduced left ventricular systolic function, mitral regurgitation, aortic root dilation, mild aortic regurgitation, diffuse dilation of coronary artery lumen, or coronary artery aneurysms

  • – Limited usefulness for measurement of coronary aneurysms after the ...