Kawasaki Disease



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Kawasaki Disease

Supplemental lab criteria:

  • Platelets >/= 450k, WBC >/= 15k, Anemia for age, Elevated ALT, UA > 10 wbc/hpf, Albumin =/< 3
Inclusion Criteria
  • Fever > 5 days PLUS 4 out of 5 criteria:
  • Bilateral bulbar conjunctival injection
  • Oral mucous membrane changes (fissured lips, strawberry tongue, erythematous pharynx)
  • Peripheral extremity changes (edema of hands/feet, erythema of palms/soles, desquamation)
  • Cervical lymphadenopathy (one node > 1.5 cm in diameter)
  • Polymorphous rash
Exclusion Criteria
  • Any other alternative explanation for symptoms
  • Does not meet 4 out of 5 additional criteria

Process consistent with KD?

Obtain CRP and ESR

CRP >/= 30 mg/L (3.0mg/dL) or ESR >/= 40 mm/hr?

  • Obtain echocardiogram (ideally at day 7-10 of fever)
  • Consider sending respiratory pathogen panel
  • Seek Infectious Disease consultation

Risk of coronary aneurysm increases with:

  • Age < 1 yo or > 9 yo
  • Male sex
  • Delay in treatment > 10th day illness
  • Refractory KD with persistent fever or return of fever > 36 hours after IVIG

  • If fever resolves, no further follow-up.
  • If fever persists, repeat ECHO and consult expert. Wait to repeat ECHO until at least day 7-10 of fever.

  • Obtain echocardiogram and cardiology consultation
  • Treat: IVIG (Gammagard) 2 g/kg over 8-12 hours, and high-dose Aspirin 30 to 50 mg/kg/day (max 4g/day) divided into 4 doses
  • If flu season, obtain influenza PCR and if positive, discuss alternative antiplatelet agent
  • Discharge once afebrile x36hr after completion of IVIG
  • Switch to Aspirin 3 to 5 mg/kg q24h upon discharge
  • Schedule follow-up with pediatric cardiologist
  • Repeat echocardiogram at 2 and 6 weeks
  • Delay live virus vaccines until 11 months post-IVIG

Treatment:

  • Obtain baseline echocardiogram and cardiology consultation
  • IVIG (Gammagard) 2 g/kg IV infusion over 8-12 hours
  • Moderate-dose Aspirin 30 to 50 mg/kg/day (max 4g/day) divided into 4 doses (either 975mg (= 325mg x 3 pills) q6h or 972mg (= 81 mg chew x 12 tablets) q6h)
  • If flu season, obtain influenza PCR and if positive discuss alternative antiplatelet agent

Exudative conjunctivitis, exudative pharyngitis, discrete intraoral lesions, bullous or vesciular rash, generalized adenopathy all suggest alternative diagnosis.

  • Treatment failure is defined as fever recurrence 36hr after completion of IVIG and would require readmission (or ongoing admission) to discuss further treatment.
  • If fever recurs within 36hr of completion of IVIG, this is not considered treatment failure. however, the patient should be observed for an additional 36hr period from last fever.

Treatment of refractory KD:

  • Seek Infectious Disease consultation
  • Repeat IVIG (Gammagard) 2 g/kg over 8-12 hours
  • Consider administration of infliximab 5mg/kg IV over 2h

Consider anxiolysis or sedation for echocardiogram for optimal study.

ECHO positive if 1 of these criteria met:

  • Z-score of LAD or RCA ≥2.5
  • Coronary arteries meet Japanese Ministry of Health criteria for aneurysms
  • ≥3 other suggestive features exist (perivascular brightness, lack of tapering, decreased LV function, mitral regurgitation, pericardial effusion, or Z-scores in LAD or RCA of 2 to 2.5)

Consider diagnosis of Incomplete/Atypical KD

Differential diagnosis also includes: viruses (EBV, measles, adenovirus), group A strep (scarlet fever, toxic shock syndrome), systemic-onset JIA, etc

  • Discharge once afebrile for continuous 36hr period after completion of IVIG
  • Switch to Aspirin 3 to 5 mg/kg q24h upon discharge
  • Schedule follow-up with pediatric cardiologist
  • Repeat echocardiogram at 2 and 6 weeks
  • Delay live virus vaccines until 11 months post-IVIG

  • Follow daily for fever
  • If fever persists x 2 days, reassess patient, consider repeat labs
  • If fever resolves, evaluate for skin desquamation
  • If no peeling: no follow-up
  • If peeling: obtain ECHO to r/o coronary aneurysm

References:

1) Brian W. McCrindle, Anne H. Rowley, Jane W. Newburger, Jane C. Burns, Anne F. Bolger, Michael Gewitz, Annette L. Baker, Mary Anne Jackson, Masato Takahashi, Pinak B. Shah, Tohru Kobayashi, Mei-Hwan Wu, Tsutomu T. Saji, Elfriede Pahl and On behalf of the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young; Council on Cardiovascular and Stroke Nursing; Council on Cardiovascular Surgery and Anesthesia; and Council on Epidemiology and Prevention. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation. 2017;CIR.0000000000000484, originally published March 29, 2017

2) Newburger JN, Takahashi M, Gerber MA, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Statement for Health Professionals From the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics 114(6), Dec 2004.

3) Sundel, R. Kawasaki Disease: Clinical Features and Diagnosis. UpToDate, online article accessed 12/12/16.

4) Sundel, R. Kawasaki Disease: Initial Treatment and Diagnosis. UpToDate, online article accessed 12/12/16.

5) Sundel, R. Incomplete (atypical) Kawasaki Disease. UpToDate, online article accessed 12/12/16.




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