Kawasaki disease
(Redirected from Kawasaki)
Background
- Also known as: mucocutaneous lymph node syndrome
- Vasculitis of unknown etiology
- Likely a combination of genetic, infectious, and regional factors
- Peaks at 18-24 months
- Rare in <4mo, >5yr
- Leading cause of acquired heart disease in children
- Coronary aneurysm more common in incomplete than in classic KD
- MIS-C has a number of overlapping clinical features, but appears to be a separate diagnosis at this time.
Vasculitis Syndrome Types
- Large vessel
- Takayasu arteritis
- Giant cell arteritis (temporal arteritis)
- Medium-vessel
- Kawasaki disease
- Polyarteritis nodosa
- Thromboangiitis obliterans (Buerger's disease)
- Primary angiitis of the central nervous system
- Small-vessel
- Henoch-Schönlein purpura
- ANCA-associated vasculitides
- Granulomatosis with polyangiitis (Wegner's)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
- Microscopic polyangiitis
- Cutaneous leukocytoclastic angiitis (“hypersensitivity vasculitis”)
- Essential cryoglobulinemia, cryoglobulinemic vasculitis due to hepatitis C
- Behçet's disease]
- Secondary vasculitides and other/miscellaneous
- Drug-induced vasculitis
- Serum sickness
- Vasculitis associated with other rheumatic diseases (e.g. SLE)
Clinical Features
- Fever that is high, abrupt. Anyone with a fever >5 days should be considered for a Kawasaki workup.
- Rash accompanies onset of fever
- Often seen in perineum. Presents as erythema and desquamation
- Later there are macular and morbilliform lesions on the trunk, arms, and legs
- No vescicles or bullae
- Maculopapular most common; vesicles not seen
- Conjunctivitis
- Bilateral and non-exudative in 90% of patients
- Anterior uveitis with cell and flare in 70% of patients but is more specific and suggestive of KD
- Mucositis: Cracked lips and strawberry tongue. Vesicles or ulcers suggest a pathology other than KD
- Edema of the hands and feet
- Desquamation around the nails in ~90%
- Lymphadenopathy is the least consistent finding in 50-75% of children
- Usually anterior cervical
- Less common in those under one year of age
- Cardiac complications develop early on
- Coronary artery aneurysm development is most prevalent as fever lessens
- KD shock syndrome (7%)
Associated Symptoms
- Cardiac
- Coronary aneurysm
- Most develop during 3-4th week of illness
- May lead to MI (leading cause of death)
- Myocarditis/pericarditis
- Pericardial effusion
- LV dysfunction
- Valvular dysfunction
- Dysrhythmias
- Coronary aneurysm
- Aseptic meningitis
- Urethritis
- Anemia
- RUQ pain, large gallbladder (hydrops on US)
Differential Diagnosis
Pediatric fever
- Upper respiratory infection (URI)
- UTI
- Sepsis
- Meningitis
- Febrile seizure
- Juvenile rheumatoid arthritis
- Pneumonia
- Acute otitis media
- Whooping cough
- Unclear source
- Kawasaki disease
- Neonatal HSV
- Specific virus
Erythematous rash
- Positive Nikolsky’s sign
- Febrile
- Staphylococcal scalded skin syndrome (children)
- Toxic epidermal necrolysis/SJS (adults)
- Afebrile
- Febrile
- Negative Nikolsky’s sign
- Febrile
- Afebrile
Evaluation
Work-Up
- CBC
- Labs
- LFTs- elevated
- ESR, CRP- elevated
- Blood culture
- Urinalysis
- ECG
- Viral panel: A positive viral PCR does not exclude KD
- 42% of children with KD had a +viral respiratory panel on initial presentation
- TTE (Coronaries, LV, valves)
- Consider if f ESR≥ 40, CRP≥ 3, or if desquamation occurs
- Red Top "Kawasaki Serum to CBR"
Evaluation
- Clinical diagnosis
Classic Kawasaki Disease | Fever for 5 days or more plus four of the following symptoms |
1. Bilateral nonexudative conjunctivitis | |
2. Mucous membrane changes (erythema, peeling, cracking of lips, "strawberry tongue," or diffuse oropharyngeal mucosal erythema) | |
3. Changes of the extremities (erythema or swelling of hands/feet, peeling of finger tips/toes in the convalescent stage) | |
4. Rash | |
5. Cervical adenopathy (more than one node >1.5 cm unusually unilateral anterior cervical) | |
Incomplete Kawasaki Disease | Fever for 5 days and two to three clinical criteria of classic Kawasaki disease plus |
C-reactive protein 3.0 milligrams/L and/or erythrocyte sedimentation rate 40 mm/h plus positive echocardiogram or three or more of the following: | |
1. Albumin <3 grams/dL | |
2. Anemia | |
3. Elevated alanine aminotransferase (ALT) | |
4. Platelets >450,000/mm3 7d after fever onset | |
5. White blood cell count >12,000/mm3 | |
6. Pyuria |
Management
Assess risk for IVIG resistance (There are 4 scoring systems):
Scoring System | Nation | Enrolled patients | IVIG resistance | Sensitivity | Specificity | Risk Factors | Point | Predicted risk (score) |
---|---|---|---|---|---|---|---|---|
Egami | Japan | 320 | 41 | 78 | 76 | Age <6 mo | 1 | Low risk (0-2) |
Illness <4 days | 1 | |||||||
CRP 8+ mg/dL | 1 | High risk (3+) | ||||||
ALT 80+ mg/dL | 2 | |||||||
Platelets < 300 | 1 | |||||||
Kobayashi | Japan | 528 | 148 | 86 | 68 | Age <12 mo | 1 | Low risk (0-3) |
Illness days <4 days | 2 | |||||||
CRP >10 mg/dL | 1 | High risk (4+) | ||||||
ALT >100 mg/dL | 2 | |||||||
Platelets < 300 | 1 | |||||||
Na <133 | 2 | |||||||
Neutrophils >80% | 2 | |||||||
San Diego | US | 362 | 60 | 73.3 | 61.9 | Illness <4 days | 1 | Low risk (0-1) |
GGT 60+ IU/L | 1 | |||||||
zHgb < -2 | 1 | High risk (2+) | ||||||
Bands >20 | 2 | |||||||
Formosa | Taiwan | 248 | 29 | 86.2 | 81.3 | Lymphadenopathy | 1 | Low risk (0-2) |
Neutrophils >60% | 2 | High risk (3+) | ||||||
Albumin <3.5g/dL | 1 |
- IVIG 2gm/kg over 12hr
- Requires inpatient monitoring for allergic reaction
- May increase ESR as IGs are positively charged which increases RBC sedimentation[1]
- Aspirin
- 30-100mg/kg/day divided 4 times a day is often used
- Has not been shown to prevent coronary artery aneurysms
- 3-5mg/kg/day is appropriate for anti-platelet activity
- Reye Syndrome has not been reported in association with KD treatment with aspirin
- Steroids
- For high-risk patients
- Decreases CAA formation if started with IVIG
- Using it as a rescue for IVIG-resistant patients is less effective
Disposition
- Admit
- Follow-up cardiac evaluation for coronary aneurysm screening
See Also
External Links
References
1. PMID: 24678327