UMEM Educational Pearls

Title: Incomplete Kawasaki Syndrome (submitted by Dr. Sarah Miller)

Keywords: Kawasaki, vasculitis, fever, (PubMed Search)

Posted: 11/25/2011 by Mimi Lu, MD
Click here to contact Mimi Lu, MD

Most of us are at least vaguely familiar with Kawasaki Syndrome as an acute vasculitis of small and medium-sized vessels, predominantly occurring in patients aged 6 months to 5 years.

Classic Kawasaki is diagnosed by fever for greater than 5 days plus 4 out of 5 classic signs.

Mnemonic: “CRASH and burn”
Conjunctivitis (bilateral and nonexudative)
Rash (polymorphous, ie can look like anything)
Adenopathy (cervical, usually greater than 1.5cm and usually unilateral)
Strawberry tongue or other oral changes (lip swelling/fissuring/erythema/bleeding, oropharyngeal hyperemia)
Hands and feet (induration and erythema, desquamation is a late sign)
Burn = fever lasting for >5 days

But what about an 8 month-old with 6 days of fever plus nonexudative conjunctivitis, unilateral cervical adenopathy and a diffuse maculopapular rash? Send some labs!

Incomplete Kawasaki is defined as fever for >5 days with 2 or more of the classic findings plus elevated ESR (>40mm/hr) and CRP (>3.0mg/dL). It is most common in infants under 12 months of age.

Disposition for the 8 month-old?

If ESR and CRP are not elevated, discharge to home with f/u in 24 hours to re-evaluate symptoms and for repeat labs if fever persists.
If ESR and CRP are elevated, the child needs an echo to evaluate for coronary artery aneurysms.

If the echo is normal, follow up in 24-48 hours and will need a repeat echo if fever persists.

TREAT kids with IVIG and aspirin (which generally means admission) if echo is positive, or with normal echo and the presence of 3 or more supplemental criteria:

Anemia for age
Elevated ALT
Albumin<3.0mg/dL,
Sterile Pyuria (>10 WBC/hpf)
Platelets >450K after 7 days
WBC >15,000

References:

1) Falcini F, Capannini S, Rigante D. Kawasaki syndrome: an intriguing disease with numerous unsolved dilemmas. Pediatric Rheumatology 2011;9:17

2) American Academy of Pediatrics. Kawasaki Disease. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2009 Report of the Committee on Infectious Diseases. 28th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2009.

Attachments

1111251332_2009_Red_Book.doc (384 Kb)