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What it is: - Acute self-limited vasculitis of childhood - Diagnostic Criteria: at least 4/5 of following in addition to fever >5days o Fever: lasting greater than or equal to 5 days. o Bilateral nonexudative conjunctivitis o Oral mucus membrane changes strawberry tongue, erythema and cracking of lips , sloughing o of filiform papillae and denuding of inflamed glossal tissue o Changes in extremities usually last manifestation to appear. Indurated edema of dorsum of hands and feet, diffuse erythema of palms or soles, edema of hands or feet, periungual desquamation o Polymorphous rash typically begins as perineal erythema and desquamation, followed by lesions of trunk and extremities o cervical lymphadenopathy least consistent feature of KD. Absent in 50-75% of children w/ dz. - 15-25% coronary artery aneurysms or ectasia ischemic heart dz or sudden death Interesting facts - one of most common vasculitides of childhood - Etiology unknown most likely infectious - MCC of acquired heart disease in children in industrialized nations - Mainly 6mo 5 yrs - Inflammation of coronary arteries is most clinically significant aspect of this process - Commonly confused w/ infectious exanthems of childhood. Pathophysiology Theory - Endothelial cell antigens become targets - Infectious agent travels through bloodstream and infects amny organs and tissues. Immune response targets these sites Sx - Ubiquitous infectious agent produces asymptomatic infxn but in genetically predisposed people, symptoms result. Management - (1) Single dose 2g/kg IVIG given over 10-12 hrs + (2)High Dose aspirin (80-100 mg/kg/day in four divided doses) - Undergo baseline echocardiogram early in hospitalization. Remain in hospital until afebrile & stable for at least 24 hrs to ensure no need for retreatment - Continue low dose aspirin (3-5mg/kg once daily) until echocardiograms at 2 wks and 6-8wks after illness onset are normal. 6-12 month follow up echo is performed at many centers, but probably unnecessary

CRP correlates w/ recurrent infxn. ESR cannot be used after IVIG has been administered because its artificially elevated following IVIG. What to look for after Tx? o CRP, loss of inflammatory signs and defervescence Long term management is based upon coronary artery status o Risk level 1 no evidence of coronary abnormalities at any time via echo o Risk level 2 transient coronary ectasia or dilatation o Risk level 3 isolated (solitary) small-medium size aneurysm in one or more coronaries o Risk level 4 1 or more large or giant aneurysm or multiple smaller or complex aneurysms w/out obstruction o Risk level 5 those w/ coronary obstruction and or ischemia

Treatment Pearls - IVIG and Aspirin when administered in first 10 days of illness reduces prevalence of coronary artery abnormalities from 25-30% in untreated individuals to 3-5% in treated pts - 10-15% of children do not respond to first dose IVIG + Aspirin. 2 nd dose IVIG is often effective. - Pts refractory to 2 doses IVIG + aspirin o Option 1: IV methylprednisolone daily for 3 days o Option 2: IVIG 3rd dose o Option 3: Single dose infliximab May predispose to TB and lymphoma - What to do if allergic to aspirin? o Clopidogrel (Plavix)

Leading cause of acquired heart disease in children 1st) Kawasaki dz 2) acute rheumatic fever What lab can you use to help monitor for recurrence Reyes Syndrome

treatment using these guidelines. Thus, these recommendations should be applied only as intended, in cases in which an experienced clinician considers KD to be a probable diagnosis. Treating children at risk even though the diagnosis is uncertain should result in fewer children with incomplete KD subsequently developing coronary artery aneurysms. This was demonstrated in a study that evaluated the performance of the 2004 AHA/AAP criteria in 195 children at four centers, 58 of whom developed coronary artery aneurysms despite failing to meet criteria for KD [6]. Fifty-three of these children had atypical KD and would have received IVIG at presentation according to the algorithm. Two of the remaining five patients would have received IVIG after further monitoring. Overall, application of the 2004 AHA/AAP guidelines would have led to IVIG treatment of 97 percent of the children who developed aneurysms. The corresponding number of children without KD who would have received unnecessary IVIG treatment was not calculated. In any event, as long as the disadvantages of undertreating appear to outweigh the disadvantages of overtreating, some children who have a condition that mimics KD will receive unnecessary intravenous immune globulin (IVIG). Thus, continued consideration of alternative diagnoses in atypical cases is essential despite initial response to IVIG. This is particularly important in children who fail to respond or respond only incompletely to IVIG.

It is important to recognize that as long as the diagnosis of KD is based upon clinical criteria, it will remain an imperfect undertaking. Although the above recommendations reflect the consensus of experts in the field, they are not based upon firm data, and they have not been validated. Ultimately, they reflect the bias in favor of treatment in uncertain cases that has developed as a result of having an effective and safe therapy. Children with a variety of other febrile conditions may receive IVIG