Emergency Department and Inpatient Clinical Pathway for Evaluation/Treatment of Children with Kawasaki Disease or Incomplete Kawasaki Disease

Consider KD for:
Patients with ≥ 3 days of fever and any principal clinical features of KD
or
Infants ≤ 6 mos with ≥ 7 days of unexplained fever
Adapted from 2017 AHA KD Guidelines
Clinical decision-making should be individualized to specific patient circumstances
 
 
Team Assessment
 
 
 
 
 
 
Complete Kawasaki Disease
  • Fever ≥ 5 days and ≥ 4 principal clinical features
  • or
  • Fever ≥ 4 days and 5 principal clinical features
  • Evaluate as clinically indicated
  • Consider pitfalls in KD diagnosis
  • Admit/discharge as clinically indicated
 
 
Admit and Treat
 
 
 
 
 
 
  • Admit
  • Evaluation as indicated
  • Consider treatment
Review Pathway for possible MIS-C Consultation as needed to determine if further labs, imaging
Discharge
Follow up in 24 hrs
 
 
Initial treatment for KD includes IVIG, ASA (high or low dose), +/- Steroids
Send save our specimen
testing prior to treatment with IVIG
 
 
 
 
Age ≤ 6 mos
Age > 6 mos
 
 
 
 
Monitor Response to Treatment
  • Review echo results with Cardiology
  • Further management based on echo results and initial treatment response
 
 
 
 
  • Discuss all abnormal echocardiogram results with Cardiology (Z-score of ≥ 2.5)
  • Infliximab Therapy
  • Principal Clinical Features of KD
    • May not all be present at the same time
  • Oral changes
    • Erythema and cracking of lips, strawberry tongue, and/or erythema of oral and pharyngeal mucosa
  • Conjunctivitis
    • Bilateral bulbar conjunctival injection without exudate
  • Rash
    • Maculopapular, diffuse erythroderma, or erythema multiforme-like
  • Extremity changes
    • Erythema and edema of the hands and feet in acute phase and/or periungual desquamation in subacute phase
  • Lymphadenopathy
    • Cervical lymphadenopathy (≥ 1.5 cm diameter), usually unilateral
See AHA Guidelines page e935, Figure 2. Clinical features of classic Kawasaki disease
  • Pitfalls in KD Diagnosis
  • Consider KD for:
    • Infants < 6 mos w/ prolonged fever
      and irritability
    • Infants with prolonged fever and unexplained aseptic meningitis
    • Infants/children with prolonged fever and any of the following:
      • Unexplained or culture negative shock
      • Cervical lymphadenitis unresponsive to antibiotic therapy
      • Retropharyngeal or parapharyngeal phlegmon unresponsive to
        antibiotic therapy
  • Documented viral or bacterial (e.g., strep) may co-exist in patients with KD
  • KD with Shock or
    KD with Macrophage Activation Syndrome
  • Consult Rheumatology and Cardiology
  • Timely management with IVIG and
    additional treatments
  • Consider ICU consult and/or management