Chapter 12. Rheumatology

Binita R. Shah

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AMA Citation
Shah BR. Shah B.R. Shah, Binita R.Chapter 12. Rheumatology. In: Shah BR, Lucchesi M, Amodio J, Silverberg M. Shah B.R., Lucchesi M, Amodio J, Silverberg M Eds. Binita R. Shah, et al.eds. Atlas of Pediatric Emergency Medicine, 2e New York, NY: McGraw-Hill; 2013. http://accesspediatrics.mhmedical.com/content.aspx?bookid=577&sectionid=42532498. Accessed April 25, 2018.

MLA Citation
Shah BR. Shah B.R. Shah, Binita R.. "Chapter 12. Rheumatology." Atlas of Pediatric Emergency Medicine, 2e Shah BR, Lucchesi M, Amodio J, Silverberg M. Shah B.R., Lucchesi M, Amodio J, Silverberg M Eds. Binita R. Shah, et al. New York, NY: McGraw-Hill, 2013, http://accesspediatrics.mhmedical.com/content.aspx?bookid=577&sectionid=42532498.

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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

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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)
1. Skin rash: polymorphic (morbilliform, maculopapular, scarlatiniform, or erythema multiforme–like); nonvesicular; commonly seen on trunk and extremities
1. Mucous membrane changes (at least one of the following): erythematous or fissured lips; erythema of buccal mucosa and pharynx; “strawberry” tongue
1. Conjunctivitis: bilateral bulbar involvement; nonexudative
1. 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)
1. 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.

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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.)