Atlas of Pediatric Emergency Medicine, 2e

Chapter 12. Rheumatology

Binita R. Shah

Clinical Summary

Kawasaki disease (KD; also known as mucocutaneous lymph node syndrome) is an immune-mediated disorder of unknown etiology that occurs in genetically predisposed children leading to endothelial cell injury and vasculitis of mainly medium-sized arteries (predilection for coronary arteries). Small arterioles, larger arteries, capillaries, and veins are affected to a lesser extent. KD occurs in all ethnic groups; however, incidence is highest in East Asia and in children of Asian descent. Clinical and epidemiologic features strongly support an infectious etiology. About 80% of patients are <5 years of age (peak: between 18 and 24 months); about 90% of patients are <8 years. There is no specific test for KD; diagnosis is based on clinical criteria summarized in Table 12.1. Other findings include extreme irritability, aseptic meningitis (50%), urethritis (sterile pyuria, 70%), hepatic dysfunction (40%), hydrops of gallbladder, diarrhea, vomiting, abdominal pain, arthritis, or arthralgia (knees, ankles, hips), uveitis, pneumonitis, testicular swelling, peripheral gangrene, erythema, or induration at bacille Calmette-Guérin inoculation site.

Table 12.1 – Clinical Features of Kawasaki Disease

View Table||Download (.pdf)

Table 12.1 – Clinical Features of Kawasaki Disease

Diagnosis is established clinically by:

Presence of fever and at least 4 of the 5 criteria listed below, or
Presence of fever and at least 3 of the 5 criteria listed below, and evidence of coronary artery abnormalities
1. Fever: high and unremitting (lasting 5 days or more, unresponsive to antipyretics and antibiotics)

Skin rash: polymorphic (morbilliform, maculopapular, scarlatiniform, or erythema multiforme–like); nonvesicular; commonly seen on trunk and extremities

Mucous membrane changes (at least one of the following): erythematous or fissured lips; erythema of buccal mucosa and pharynx; “strawberry” tongue

Conjunctivitis: bilateral bulbar involvement; nonexudative

Changes in distal extremities (at least one of the following): erythema of palms or soles; indurative edema of hands or feet; periungual desquamation of fingers and toes (1–3 weeks after onset of illness)

Cervical lymphadenopathy (least constant finding): unilateral; at least one node 1.5 cm or larger in diameter; nonpurulent

Clinical phases (after onset of illness):

Acute febrile phase: up to 1 to 2 weeks (fever and other acute signs)
Subacute phase: up to 2 to 4 weeks (fever abated, desquamation, thrombocytosis, development of coronary aneurysms)
Convalescent phase: up to 6 to 8 weeks (illness disappears, ESR, C-reactive protein return to normal)

Figure 12.1 ▪ Kawasaki Disease.

View Full Size||Download Slide (.ppt)

A 3-year-old child with a history of high fever of 6 days’ duration associated with diffuse erythematous maculopapular rash, red lips, and bilateral conjunctival injection. (Photo contributor: Binita R. Shah, MD.)