INCOMPLETE KAWASAKI DISEASE
5 year old female presenting to the Emergency Department via EMS with increased work of breathing, cough, wheezing and a rash on the arm that began 2 hours prior to arrival. Also with fever to 102.7 at home.
No albuterol available at home, but did receive a nebulizer treatment by EMS on route to the hospital.
No history of recent travel or trauma. No known sick contacts. Denies nausea, vomiting, nasal congestion or abdominal pain.
Vaccines up to date.
Past medical history significant for asthma, eczema and seasonal allergies.
Upon initial assessment in the ED, patient found to be ative and alert without any distress. Eyes and ears normal. Neck supple. No lymphadenopathy noted. Lung exam significant for bilateral expiratory wheezing without retractions. Skin exam reveals fine papular rash (sandpaper like) on trunk and extremities.
Vital signs: T 37.4 HR 140 BP 109/59 RR 40 O2 100%
Respiratory symptoms improved with albuterol. Patient found to be rapid strep positive, treated with LA Bicillin and discharged home.
3 days later, patient returned to the ED with fevers up to 39. No other change in symptoms
Vital signs: T 39.2 HR 135 RR 26 O2 98%
Physical exam now significant for erythema of the posterior pharynx and bilateral anterior cervical lymphadenopathy. Also noted was that patient was crankier, but consolable.
Patient discharged home again with diagnosis of resolving strep pharyngitis.
Patient returned to the ED 4 days later with continued daily fevers up to 40 C. Continued rash and now with cough and nasal congestion.
Vital signs: T 36.5 HR 123 BP 90/60 RR 23 O2 98%
Physical examination on this day was significant for hepatomegaly and peeling of the skin around the eyes. (otherwise, previous findings of rash and lymphadenopathy were still present)
Labs:
CBC - WBC 14.2 (45% N, 9% L, 15% Bands); Platelets 465
LFTs - LDH 338; GGT 151; Protein 6.7; T Bili 12; D Bili 9; Alk Phos 509; ALT 90; AST 93; Albumin 3.3; Amylase/Lipase wnl.
Electrolytes wnl
UA - WBC 11-25; Blood neg; Nitrite neg; Bili large
CRP - 45; ESR - 60
What is the diagnosis?
Kawasaki Disease is one of the most common vasculitidies of childhood. Peak age is 18-24 months (50% < 2 years of age; 80% < 5 years of age).
Less commonly seen in those over 8 years old, but has been reported up until 12 years.
Fever and acute inflammation for 10-12 days.
20% of untreated patients will develop coronary artery abnormalities.
Diagnostic Criteria:
- Fever for more than 4 days with no other cause and 4/5 other criteria
- Bilateral bulbar conjunctival injection
- Oral mucous membrane changes (strawberry tongue; red lips; cracked liips; fissures of mouth)
- Peripheral extremity changes (edema of hands/feet [acute]; periungal desquamation [convalescent])
- Polymorphous rash (morbilliform; scarlatiniform; maculopapular; EM)
- Cervical adenopathy (at least 1 node > 1.5 cm; unilateral)
Those less than 1 year old or greater than 5 years are more likely to have atypical presentation. "Incomplete" Kawasaki has 5 or more days of fever with 2 or more of the noted criteria. Supprtive lab data (ESR > 40; CRP > 3). Coronary artery disease confirms the diagnosis. Normal early echo does not exclude the diagnosis.
Associated Findings:
-Urethritis with sterile pyuria (70%)
-Hepatic dysfunction (40%)
-Anterior uveitis (25-50%)
-Arthritis or arthralgia (10-20%)
-Meningismus with CSF pleocytosis (25%)
-Gallbladder hydrops (<10%)
-Pericardial effusion (<5%)
-Myocarditis (<5%)
-Irritability
-Desquamation in the diaper area
Conclusion:
Cardiology and Infectious Disease consults were performed. The patient was admitted to the hospital and IVIG was held pending echocardiogram in the am.
Echocardiogram was normal, however abdominal ultrasound revealed hydrops of the gallbladder.
Patient was started on high-dose aspirin and IVIG.
Fever resolved within 48 hours of admission/treatment and patient was discharged home with both cardiology and GI follow-up.
EVIDENCE
Given no gold-standard for diagnosis of Kawasaki Disease, it must be remembered that the above algorithm is not evidence based. It was formed from the concensus of an expert committee.
Patients with fever of 5 days or longer with less than 4 principal features can be diagnosed when coronary artery disease is detected by 2-dimensional echocardiography or coronary angiography.
In those with 4 or more principal features, the diagnosis can be made on day 4 of illness.
Kawasaki Disease should be considered in any child with unexplained fever for 5 or more days associated with any of the principal clinical features of the disease.
Because of imperfect performance in scoring systems used to predict who will develop aneurysms, all children diagnosed with Kawasaki Disease should be treated with IVIG.
High dose aspirin is used in the treatment of the acute pase of illness (80-100mg/kg/day divided Q6hrs). High dose ASA and IVIG appear to have an additive antiinflammatory effect. The duration of high-dose aspirin administration is not standardized and tends to vary by institution.
When high dose aspirin is stopped, low dose is begun (3-5mg/kg/day). This is usually maintained for 6-8 weeks of illness in those with no evidence of coronary disease. In those with coronary disease, it may be continued indefinitely.
