Morning Report 7/13/09

Acute febrile vasculitic syndrome of early childhood

Affecting all blood vessels in the body but mostly medium and small vessels with a preferential involvement of the coronary arteries.

Exact etiology unknown but thought to be infectious in nature

Immune response thought to be oligoclonal or antigen driven

Race: Japanese > Blacks, Polynesians, Filipinos > Whites

Gender: Male:Female~ 3:2 Age:

90-95% <10years old Peak incident 18-24months

Presence of 5 or more days of fever + 4 or more of the 5 principle clinical features

Arthritis/arthralgia Irritability Diarrhea, Vomiting, Abdominal Pain Hepatomegally, Jaundice Pleural Effusions, infiltrates Stiff Neck secondary to aseptic meningitis

Children with unexplained fever for more than 5 days associated with 2-3 of the principle clinical features

More common in young infants May be supported by laboratory evidence

of systemic inflammation

Viral Infections (Measles, adenovirus, enterovirus, EBV)

Scarlet Fever Staphylococcal scalded skin syndrome Bacterial cervical lymphadenitis Rocky Mountain Spotted Fever Leptospirosis

Moderate to high WBC count with left shift

Anemia Elevated ESR, CRP Thrombocytosis Mild-Moderate elevation in transaminases Sterile Pyuria

Toxic Shock Syndrome Drug Hypersensitivity Steven-Johnson syndrome Juvenile idiopathic arthritis Juvenile Polyarteritis Nodosa Mercury hypersensitivity reaction

Echocardiogram is critical for the evaluation of all patients suspected of having KD. Baseline echo during acute stage to r/o coronary

artery aneurysms and evidence of myocarditis, valvulitis, or pericardial effusion

Echo should be repeated in 2nd-3rd week of illness and again 1 month after (or once all lab values normalize)

Prior to treatment 20-25% of patients had Cardiac involvement with mortality rate 0.1-2%

With IVIG risk reduced to 5%

Standard therapy is IVIG with Asprin During the acute phase of illness

IVIG (2gm/kg) and Asprin 80-100mg/kg /day

Continue high dose asprin until day 14 of illness if still afebrile Continue asprin 3-5mg/kg/day until no

evidence of coronary changes by 6-8 weeks

~10% fail to respond to initial IVIG therapy (persistence of fever after 36hrs)

Retreatment with IVIG at same dose recommended

3rd dose IVIG Pulse Steroids (Methylprednisolone mg/kg

for 2-3 hours qday x3days) Infliximab (monoclonal ab against tumor

necrosis factor) Cyclophosphamide Methotrexate

MI caused by thrombotis occlusion of abnormal coronary artery Is principle cause of death

Usually occurs within first year Children at high risk need frequent ECHO

evaluations Small solitary aneurysms-long term asprin

therapy Giant aneurysms or multiple complex

aneurysms-long term antiplatelet therapy and anticoagulation

Primary surgical management is coronary artery bypass graft