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
Infants ≤ 6 months with ≥ 7 days of unexplained fever
Adapted from 2017 AHA KD Guidelines
Clinical decision-making should be individualized to specific patient circumstances
  • FLOC/RN Team Assessment
  • History & Physical, Clinical Criteria
    • Assess for presence of clinical criteria at any time during current febrile illness
  • Laboratory Testing
    • Lab testing if H&P consistent with complete or incomplete KD.
    • Consider lab testing if 3 days of fever and strong clinical suspicion for KD.
Complete Kawasaki Disease
  • Use Incomplete Kawasaki Disease Algorithm to determine need for further evaluation and treatment
  • 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 for further evaluation
Possible treatment
Review Pathway for possible MIS-C Consultation as needed to determine if further labs, imaging
Follow up in 24 hours
Initial Treatment
Initial treatment for KD includes IVIG, ASA (high or low dose), +/- Steroids
Send save our specimen and SARS-CoV-2 serology
testing prior to treatment with IVIG
Age ≤ 6 months
Age > 6 months
  • Intravenous Immunoglobulin
  • Aspirin -
    • High dose x 24 hours
    • Low dose after 1st 24 hours
  • Echo within 24 hours
  • Consults —
    • Cardiology
    • During COVID-19 pandemic consult Infectious Diseases for all KD patients
Monitor response to treatment
Review echo results, discuss with cardiology
Further management based on echo results and initial treatment response for all ages
Discuss all abnormal echocardiogram results with Cardiology.
  • 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 months 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.
  • Suspected MIS-C
  • Consult ID, Rheumatology
  • Consider DIRT and Cardiology prn
  • Consider ICU consult and/or management
Posted: January 2018
Revised: February 2018, January 2019, March 2020, May 2020, July 2020
Authors: D. Whitney, MD; K. Dorland, BSN; J. Beus, MD; J. Brothers, MD; L. Buckley, MD; S. Burnham MD; D. Campeggio, MSN; K. DiPasquale, MD; H. Ghanem MD; J. Hart MD; J. Lavelle, MD; C. Law PharmD; S. Natarajan, MD; J. Ronan, MD; V. Scheid, MD; S. Swami, MD; H. Baxter, CRNP
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