KAWASAKI DISEASE

Chapter 191
Nelson’s Textbook of Pediatrics – 20th Edition
 KAWASAKI DISEASE

• aka mucocutaneous lymph node syndrome / infantile polyarteritis nodosa

• Acute febrile illness of childhood
• Systemic inflammatory disorder manifesting as vasculitis with a predilection for
the coronary arteries
 ETIOLOGY

• Cause: unknown
• Probable causes:
• Infectious – young age group, wavelike geographic spread, self-limited nature of
febrile illness, clinical features, infrequent occurrence in <3mos old, rarity of adult
cases
• Genetic – higher risk in Asian children, associations between polymorphisms in
ITPKC gene = increased susceptibility to KD, association of SNP in HLA class II
 EPIDEMIOLOGY

• Seen worldwide
• Highest incidence in Asian children
• Early childhood: <5 years
• M>F
• Leading cause of acquired heart disease in children in most developed countries (US, Japan)
• Coronary artery abnormalities
• 20-25% of untreated children
• <5% in children treated with IVIG
• Risk factors: race (Asian, Pacific Islander, Hispanic), young age, male, persistent fever, poor response to
IVIG, laboratory abnormalities (neutrophilia, thrombocytopenia, transaminitis, hyponatremia,
hypoalbuminema, elevated BNP and CRP)
 PATHOLOGY

• affects medium-sized arteries:

coronary arteries (most common)
• Other arteries: popliteal, brachial
arteries
• 3 phases of arteriopathy
• 1st phase – neutrophilic
necrotizing arteritis
• First 2 weeks of illness
• Begins in the endothelium and moves
through the coronary wall = saccular
aneurysms
 PATHOLOGY

• 2nd phase - subacute/chronic vasculitis

• Lymphocytes, plasma cells, eosinophils
• Results in fusiform aneurysms
• Lasts for weeks to years
• 3rd phase – luminal myofibroblastic
proliferation
• Affected vessels develop smooth muscle
cell myofibroblasts = progressive stenosis
• Forms thrombi and obstruct blood flow
CLINICAL MANIFESTATIONS
 CLINICAL COURSE

• 3 clinical phases
• Acute febrile phase – fever and other acute signs of illness (1-2 weeks)
• Subacute phase – desquamation, thrombocytosis, development of CAA (lasts 3
weeks)
• Highest risk of sudden death in patients whom aneurysms have developed
• Convalescent phase – all clinical signs of illnesses disappears, ESR normalizes
(6-8 weeks after onset of illness)
LABORATORY & RADIOLOGIC FINDINGS

• 2D echocardiography – most useful to monitor

CAA
• Should be performed at diagnosis and after 2-3wks
of illness
• If normal, repeat study 6-8 week after onset of illness
 DIAGNOSIS

• Classic KD
• Atypical or incomplete KD
DIFFERENTIAL DIAGNOSIS