Kawasaki disease An acute, febrile, vasculitic disease of early childhood (most commonly <5). Incidence: 19 per 100,000 annually in US Pathophysiology: Epidemiological studies indicate an infectious agent that has not been identified, but also suggest some underlying genetic susceptibility. Pathology of the affected arteries (medium-sized muscular arteries) demonstrates involvement of all layers of the artery. Diagnosis: Fever >5 days is required, accompanied by 4 of these 5: - Conjunctivitis (90%) - Bilateral conjunctival injection. Will be nonexudative, and spares the limbus - Rash – polymorphous but not vesicular. Most often generalized, but can be limited to groin & legs. - Adenopathy (75%) – usually unilateral, a single large cervical node measuring ~1.5cm - Strawberry tongue/oropharyngeal changes. Can include lip fissures, erythema, or crusting) - Hands – reddening or edema of the hands and feet, followed by desquamation of the fingertips and toe tips. Or can see “Beau lines” – a transverse groove across the fingernails and toenails Mnemonic: CRASH and Burn (the fever) Constellation not used for diagnosis can include: - Irritability - Anterior uveitis (70%) - Perianal erythema (70%) - Sterile pyuria - Myocarditis and pericarditis - Hepatic, renal and GI dysfunction Labs: There is no “gold standard” lab diagnosis, but you will often see elevated ESR, CRP, and Alpha-1 antitrypsin. Recent research has pointed to two urinary proteins – meprin A and filamin C which have higher diagnostic value and could be potential biomarkers. Management/Treatment Goals: Prevent coronary artery disease, and control symptoms. - Perform echocardiograms: o At time of diagnosis o 2 weeks and 6-8 weeks after onset of illness - Symptom control: o High doses of IVIG, and a high dose of aspirin followed by low-dose aspirin - Other options include non-aspirin anticoagulants, Sources: Scheinfeld, Kawasaki Disease, Medscape, Aug 14, 2017; Takahashi K, Pathogenesis of Kawasaki Disease, Clin Exp Immunol, 2011; Diagnosis, treatment, and long-term management of Kawasaki disease, American Heart Association Prepared by Dave Carlson. Rady Children’s Hospital Inpatient Pediatrics seminar, 10/18/2017
