Background
Kawasaki disease (KD), also called Mucocutaneous Lymph Node Syndrome, is an acute, systemic vasculitis of small and medium-sized arteries.
“KD is characterized by systemic inflammation in all the medium-sized arteries and in multiple organs and tissues during the acute febrile phase, leading to associated clinical findings.” AHA 2017
Mainly affects patients 5 months to 5 years of age. But younger and older populations (even adults) can still have Kawasaki, particularly atypical KD which is more common in younger and older populations. “Asian and black Americans are 2.5 and 1.5 times more likely to develop Kawasaki disease than whites.”
Diagnosis
H&P
Clinical presentation:
Typical vs. Atypical Kawasaki.
Clinical Findings.
Diagnostic criteria (CRASH and burn the heart). Diagnosis is clinical.
Diagnostic criteria for suspected incomplete Kawasaki.
Complications: Heart abnormalities (Aneurisms) may occur in the first week. Get TTE early.
Ddx and Etiology.
Diagnostic testing
-Transthoracic Echo to r/o aneurisms.
–Supplemental laboratory criteria (not required for diagnosis) to help with atypical Kawasaki
–CRP, ESR; CBC with WBC differential; CMP (serum albumin, liver enzymes); Urinalysis (U/A), preferably clean catch.
Treatment
IVIG + high dose Aspirin. If no response, give 2nd dose with or without steroids.
Algorithm for atypical Kawasaki.
Once you’ve diagnosed atypical KD, you treat it the same way as typical KD.
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Steroids are only used as an adjunct. They can be used alone. Tx is IVIG + High dose Aspirin.
“Infants and possibly adults are more likely to present with incomplete KD. Infants are at greater risk for cardiovascular sequelae, possibly due in part to a delay in diagnosis and intervention. Thus, infants six months of age or less with unexplained fever for at least seven days should be evaluated for KD, even if they have no clinical findings of KD.” Up to date
Atypical Kawasaki
“Supplemental laboratory testing can help in the diagnosis of these atypical Kawasaki cases.
The supplemental laboratory criteria include:
- Anemia
- Cerebrospinal fluid pleocytosis
- Elevated C-reactive protein and erythrocyte sedimentation rate
- Elevated liver enzymes
- Hypoalbuminemia
- Hyponatremia
- Platelets >450,000/mm3 after 5 weeks
- Sterile pyuria
- WBCs ³15,000/mm3
Patients who have a fever for 5 days or more and 2 or 3 of the classic criteria should be treated for atypical Kawasaki’s disease if the C-reactive protein level is elevated and they have 3 or more associated laboratory abnormalities. The treatment of choice is IVIG and high-dose aspirin to reduce the risk of coronary abnormalities. Corticosteroids have been used as an adjunct in refractory cases or with IVIG, but not alone.” ABFM Critique
References / Further Reading
Circulation. 2017 Apr 25;135(17):e927-e999. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. https://www.ncbi.nlm.nih.gov/pubmed/28356445. Full version article, here.
Korean Journal of Pediatrics. 2012;55(3):83-87. Diagnosis of incomplete Kawasaki disease. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3315623
https://www.mdedge.com/ecardiologynews/clinical-edge/summary/cardiology/updated-guidance-treating-kawasaki-disease
Am Fam Physician 2015;91(6):365-371. Diagnosis and management of Kawasaki disease.
https://www.uptodate.com/contents/incomplete-atypical-kawasaki-disease