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 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
  • 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 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 with Cardiology
  • Further management based on echo results and initial treatment response
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
Evidence