As you are starting your morning ED shift, your first patient is a 2-year-old boy, previously healthy,  who is coming in with 5 days of fevers at home. Fevers have occurred daily and have been as high as 39.5ºC. His mother states that during this time he has been more fussy than usual and has been not been eating or drinking very well. Vital signs demonstrate a temperature of 38.9ºC, HR of 150, RR is 20. His BP is 110/60.  On exam, he is fussy and appears ill. His conjunctiva are injected and you appreciate limbic sparing.  Oropharyngeal exam is shown below. You also note a diffuse macular rash. What are you most concerned about?

Picture1

 

Kole A, Chandakole D. N Engl J Med 2015;373:467-467.

 

Answer: The picture above shows a strawberry tongue with fissured lips, which in conjunction with 5 days of fever, rash and bilateral conjunctival injection is concerning for Kawasaki Disease (KD). KD is an acute vasculitis of still unknown etiology affecting primarily infants and children. KD  affects coronary arteries, and without IVIG, 20% of children will go on to have coronary artery aneurysms (Newburger et al). Diagnosis of KD is primarily clinical, and requires 5 or more days of fever in conjunction with 4/5 clinical criteria (see below). For those children with persistent fever, but who lack all 4 criteria (child mentioned in case has 3), diagnosis utilizes lab and ECHO findings (listed below). In children with classic Kawasaki, as well as those with incomplete disease, the mainstay of acute management is IVIG paired with high-dose aspirin. For more information, please review the attached references.

 

Diagnostic Criteria of Kawasaki Disease (requires at least 5 days of fever)

  1. Changes in extremities: Acute: Erythema and edema of hands and feet Convalescent: Desquamation of fingertips
  2. Polymorphous exanthema
  3. Bilateral, painless bulbar conjunctival injection without exudate
  4. Changes in lips and oral cavity: Erythema and cracking of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosae
  5. Cervical lymphadenopathy (≥1.5 cm in diameter), usually unilateral

Diagnostic Criteria for children who have at least 5 days of fever and only 2-3 findings mentioned above:

  1.  Obtain Labs: CRP/ESR, CBC, LFTs, and UA
  2. If CRP < 3 mg/dl AND ESR <40
    1. If fever continues, re-evaluate
    2. If fever defervesces, no follow-up required (EXCEPTION: in children who develop desquamation, an ECHO should be obtained).
  3. If CRP ≥ 3 mg/dl and/or ESR ≥40, obtain ECHO 
    1. If > 3 supplementary lab findings (See Below), treat with IVIG and high dose ASA
    2. If < 3 supplementary Findings
      1. ECHO positive (see below for criteria) –> TREAT
      2. ECHO Negative
        1. If fever resolves, Kawasaki is unlikely
        2. If fever persists, obtain 2nd ECHO, consult specialist

Important Lab or Imaging Findings

  1. Supplementary Lab Findings (Need 3 or more)
    1. LFTs: Albumin ≤ 3 g/dL or elevated ALT (> 50 units/L)
    2. CBC: WBC ≥ 15,000 or PLT≥ 450,000 after 7 days, or anemia for age [normochromic, normocytic]
    3. UA: ≥10WBC per HPF
  2. ECHO findings
    1. Any of the Following
      1. z score of LAD or RCA ≥2.5
      2. coronary arteries meet Japanese Ministry of Health criteria for aneurysms
      3. ≥3 other suggestive features exist:
        1. perivascular brightness
        2. lack of tapering
        3. decreased LV function
        4. mitral regurgitation
        5. pericardial effusion,
        6. zscores in LAD or RCA of 2–2.5

From: AHA Scientific Statement on Kawasaki Disease 2004.

Faculty Reviewer: Erica Chung, MD

 

References

  • “Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease.” Circulation. 2004; 110(17)2747-2771
  • Son MB and Newburger JW. “Kawasaki Disease.” Pediatrics in Review. 2013; 24(4)151-161
  • Newburger JW et al. “Kawasaki Disease.” Journal of the American College of Cardiology. 2016;67(14)1738-49.