Kawasaki Disease, Emergency Medicine

Basics

Description

Acute inflammatory process involving multiple organs
Leading cause of childhood-acquired heart disease in developed countries
Vasculitis is most severe in medium-sized arteries
Acute cardiac sequelae:
- Coronary artery aneurysm:
  - Often lead to stenosis after healing
- Giant aneurysm:
  - May rupture
- Myocarditis
- Pericarditis
Stages:
- Acute (lasts 1 " “2 wk):
  - Fever
  - Oral mucosal erythema
  - Conjunctival injection
  - Erythema and edema of hands and feet
  - Cervical adenopathy
  - Aseptic meningitis
  - Hepatic dysfunction
  - Diarrhea
  - Myocarditis
  - Pericardial effusion (20 " “40%)
  - No aneurysms by ECHO
- Subacute (when fever, rash, and lymphadenopathy resolve until about 4 wk):
  - Anorexia
  - Irritability
  - Desquamation of hands and feet
  - Thrombocytosis
  - Coronary artery aneurysms (20% if untreated)
  - Risk for sudden death is highest
- Convalescent phase (about 6 " “8 wk):
  - Clinical signs are absent
  - ESR normalizes
- Epidemiology:
  - 80% of cases occur in children <4 yr old; peak at 1 " “2 yr; rare in infants <3 mo old
  - Adult cases have been reported
  - Asians are at highest risk
  - Males > females 1.5:1
Genetics:
- Possible genetic predisposition
Risks for nonresponse to standard therapy (10 " “15%):
- Elevated band count
- Low albumin level
- Abnormal initial ECHO
Risks for development of coronary artery aneurysms:
- Extremes of age
- Male gender
- Prolonged fever
- Persistent fever after treatment
- Delay in diagnosis
- Increased WBC and/or band count
- Low hematocrit
- Significant increase in CRP and/or ESR

Etiology

Unknown " ”believed to be infectious based on manifestations of disease, epidemics, and increased numbers of cases in winter and early spring
Current theory:
- Activation of immune system in response to infection
- Genetically susceptible host
- May explain why certain ethnicities have higher incidence of disease
  - More prominent in Asian countries

Diagnosis

Classic diagnostic criteria:
- Fever for 5 days + 4 of the 5 following criteria:
  - Bilateral conjunctival injection
  - Changes in oral mucosa
  - Polymorphous erythematous rash
  - Changes in hands or feet " ”edema, erythema, desquamation
  - Cervical lymphadenopathy >1.5 cm (least common)
Atypical cases can be seen without meeting diagnostic criteria
- Fever for >5 days + 2 or 3 clinical criteria with ESR >40 and CRP >3
- If >3 of the below can diagnose incomplete Kawasaki disease
  - Albumin <3
  - Anemia for age
  - ALT elevation
  - WBC > 15,000
  - Urine >10 WBC per high power field
  - Platelets >450,000 after 7 days
Thrombocytosis
- Changes in hands or feet " ”edema, erythema, desquamation
- Cervical lymphadenopathy >1.5 cm (least common)

Signs and Symptoms

History

Temperature >38.5 ‚ °C (often spiking) for at least 5 days:
- Begins abruptly and may last >2 wk
Cardiac:
- Shortness of breath
- Chest pain
HEENT:
- Eyes:
  - Conjunctivitis
  - Photophobia
- Mouth:
  - Erythema
  - Dry and fissured lips
Skin rash
Musculoskeletal:
- Arthralgia, arthritis
Neurologic:
- Extreme irritability
GI:
- Diarrhea
- Vomiting
- Abdominal pain

Physical Exam

Cardiac:
- Evidence of congestive heart failure
- Evidence of pericarditis
  - Rub
- Evidence of valvular disease
  - Murmur
HEENT:
- Eyes:
  - Bilateral conjunctival injection without exudates
  - Bulbar conjunctiva is more frequently involved than palpebral conjunctiva
  - Usually within 2 days of onset of fever and lasting 1 " “2 wk
  - Photophobia, uveitis, iritis
- Mouth:
  - Erythema, dry and fissured lips, strawberry tongue, pharyngeal erythema
- Lymph:
  - Cervical lymphadenopathy (node diameter >1.5 cm)
Neurologic:
- Irritability
- Meningismus
Skin:
- Rash, primarily on the trunk
- May be maculopapular, scarlatiniform, or erythema multiforme " “like; erythroderma
- Changes in the hands or feet " ”erythema, edema (acute phase); unwilling to bear weight
- Desquamation (subacute phase) of the tips of fingers and toes 2 " “3 wk after onset of illness
Genitourinary:
- Urethritis
- Meatitis
GI:
- Hydrops of the gallbladder

Essential Workup

Must think of the diagnosis in a febrile child with rash ‚

Diagnosis Tests & Interpretation

Lab

CBC:
- WBC " ”normally elevated with shift to left in acute phase
- Normocytic anemia
- Leukopenia and thrombocytopenia are rare
  - Suspect viral infection
  - Thrombocytopenia is a risk factor for development of coronary artery disease
Urinalysis:
- Sterile pyuria
- Proteinuria
ESR elevated from 1st wk until 4 " “6 wk
Increased C-reactive protein
CSF pleocytosis
Cultures: Negative blood, urine, CSF, throat
Increased transaminases and bilirubin

Imaging

ECHO to evaluate for coronary artery aneurysm:
- Acute phase (baseline)
- 2 " “3 wk
- 6 " “8 wk
CXR

Diagnostic Procedures/Surgery

ECG if concern about MI or pericarditis
Slit-lamp exam " ”uveitis

Differential Diagnosis

Viral infections:
- Adenovirus
- Enterovirus
- Measles
- Epstein " “Barr virus
- Rubella
- Rubeola
- Influenza
Bacterial infection:
- Scarlet fever (responds rapidly to penicillin)
- Staphylococcal scalded-skin syndrome
- Rickettsial disease, including Rocky Mountain Spotted Fever and leptospirosis
- Cervical adenitis
Immune-mediated:
- Stevens " “Johnson syndrome
- Erythema multiforme
- Serum sickness
- Connective tissue disease (i.e., Lupus)
- Other forms of vasculitis

Treatment

Pre-Hospital

ABCs
Oxygen

Initial Stabilization/Therapy

ABCs with focus on cardiovascular system ‚

Ed Treatment/Procedures

Initiate IV gammaglobulin (IVIG) and aspirin therapy:
- Do not generally need to monitor salicylate levels because of decreased absorption and increased clearance
Treatment within the 1st 10 days of illness reduces cardiac sequelae from range of 20 " “25% to range of 2 " “4%.
Cardiology consultation
Treatment of MIs as in adults

Medication

First Line

IVIG: 2 g/kg IV over 10 " “12 hr; retreatment may be required for persistent (>48 " “72 hr) or recrudescent fever:
- Requires close cardiac monitoring
- Should be started within the 1st 10 days of illness
- 3 " “4% failure rate after 2 doses
Aspirin: 80 " “100 mg/kg/d PO q6h until about day 14 when fever has resolved; then 3 " “5 mg/kg/d PO daily for 6 " “8 wk. Do not exceed 4g/24 h (peds: Do not exceed 120 mg/kg/24 h):
- Anti-inflammatory
- Antiplatelet
- Potentiates the action of IVIG
- Reduces the occurrence of aneurysms when given with IVIG
- Alternative dosing at 30 mg/kg/d during acute and subacute phases

Second Line
If no response to 2nd dose of IVIG ‚

Corticosteroids:
- Methylprednisolone 30 mg/kg over 3 hr
- May improve outcome in conjunction with IVIG

May also consider: ‚

Infliximab
Cyclosporine A
Methotrexate

Follow-Up

Disposition

Admission Criteria

Admit all patients who fulfill diagnostic criteria for Kawasaki disease
Admit toxic-appearing patients who do not yet meet the criteria for Kawasaki disease

Discharge Criteria

Nontoxic children who do not fulfill diagnostic criteria
Close follow-up is required

Issues for Referral
Cardiology consultation for all patients ‚

Pearls and Pitfalls

Prompt diagnosis and therapy can prevent coronary aneurysms in 95%
Aspirin and IVIG are mainstays of therapy
Must consider the diagnosis in febrile children presenting to the ED for multiple visits
Restrict steroids to children with 2 IVIG failures

Additional Reading

Ashouri ‚ N, Takahashi ‚ M, Dorey ‚ F, et al. Risk factors for nonresponse to therapy in Kawasaki disease. J Pediatr. 2008;153:365 " “368.
Gerding ‚ R. Kawasaki disease: A review. J Pediatr Health Care. 2011;25:379 " “387.
Harnden ‚ A, Takahashi ‚ M, Burgner ‚ D. Kawasaki disease. BMJ. 2009;338:b1514.
Kuo ‚ HC, Yang ‚ KD, Chang ‚ WC, et al. Kawasaki disease: An update on diagnosis and treatment. Pediatr Neonatol. 2012;53:4 " “11.
Newburger ‚ JW, Sleeper ‚ LA, McCrindle ‚ BW, et al. Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease. N Engl J Med. 2007;356:663 " “675.
Newburger ‚ JN, Takahashi ‚ M, Gerber ‚ MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: A statement forhealth professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics. 2004;114:1708 " “1733.
Scuccimarri ‚ R. Kawasaki disease. Pediatr Clin N Am. 2012;59:425 " “445
Tacke ‚ CE, Burgner ‚ D, Kuipers ‚ IM, et al. Management of acute and refractory Kawasaki disease. Expert Rev Anti Infect Ther. 2012;10:1203 " “1215.

Codes

ICD9

429.0 Myocarditis, unspecified
446.1 Acute febrile mucocutaneous lymph node syndrome [MCLS]
447.6 Arteritis, unspecified
420.90 Acute pericarditis, unspecified

ICD10

I51.4 Myocarditis, unspecified
I77.6 Arteritis, unspecified
M30.3 Mucocutaneous lymph node syndrome [Kawasaki]
I31.9 Disease of pericardium, unspecified

SNOMED

75053002 Acute febrile mucocutaneous lymph node syndrome (disorder)
31996006 Vasculitis (disorder)
50920009 Myocarditis (disorder)
3238004 Pericarditis (disorder)

Home

Erectile Dysfunction

Doctor123.org