Kawasaki Disease
Susa Benseler
Introduction
Kawasaki disease (KD) is an inflammatory syndrome that causes a systemic vasculitis with predilection of the coronary arteries
Etiology unclear; however, a wide variety of infections is associated with development of KD in children
Primarily affects young children, 80% < 5 years
Japan has highest incidence
Most common cause of acquired heart disease in Western countries
Kawasaki’s is a potential complication/common pathway of multiple triggers/infections; should be considered in all children with prolonged fever
Diagnostic Criteria
Clinical diagnosis based on presence of fever for at least 5 days plus 4/5 criteria
Fever ≥ 5 days plus:
Conjunctival injection/nonpurulent conjunctivitis (red eyes)
Oral changes: red, cracked lips/oral erythema, prominent follicles of lip (strawberry tongue), diffuse redness of oral cavity
Cervical lymphadenopathy (> 1.5 cm in diameter)
Polymorphous rash
Swelling/redness of hands and feet
Infants < 1 year and older children > 9 years often do not meet all criteria
Clinical Presentation
Frequency of clinical features:
Fever in all patients, mean duration 6.5 days
< 5 days of fever: 11.8%
Oral changes in 94%
Conjunctivitis in 92%
Rash in 90%
Extremity changes in 77%
Cervical lymphadenopathy in 64%
Table 40.1 Definitions | ||||||||
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May also present with:
Cardiac: hypotension, tachycardia, myocarditis, pericarditis, pericardial effusion, arrhythmia, valvulitis (< 1%), mitral regurgitation
CNS: headaches, aseptic meningitis, acute encephalitis
LN: lymph node abscess/severe adenitis, generalized lymphadenopathy
Lungs: pneumonitis, pleuritis
Abdominal symptoms: gallbladder hydrops, noninfectious hepatitis, mesenteric vasculitis, ischemic strictures, pseudo-obstruction
MSK: arthritis, myositis
Systemic vasculitis (1%)
Associated features may be presenting symptom: risk of missing diagnosis
Consider Kawasaki disease in every child with ≥ 5 days of fever
Investigations
