Table 1 Major landmarks in the evolution of Kawasaki disease in this three-year old infant

Day 1 to 9^th

Fever; maculopapular rash; lethargy

10^th day

Hospital admission; persisting fever

High CRP; chest X-ray: pneumonia; CSF: aseptic meningitis

Wide spectrum antibiotic (ceftriaxone)

14^th day

KD characteristic clinical signs, persisting fever, cyanosis of toes and blackening of halluces, reactivation of BCG scar

Doppler ultrasound: absence of blood flow in distal tibiofibular branches. Computed angio-tomography: dilatation of both iliac arteries; pulmonary thrombosis; splenic infarction. 1^st Echocardiogram: normal coronary arteries. High levels of inflammatory markers.

Reintroduction of wide- spectrum antibiotics (cefepime & vancomycin); anticoagulant therapy: enoxaparin; alprostadil; 1^st administration of IVIG & ASA (80 mg/kg/day),

17^th day

Persisting fever

Inflammatory markers still high; 2^nd echocardiogram: significant dilatation of coronary arteries

ASA decreased to (80 mg/kg/day); Started three daily doses of 30 mg/kg/day of methylprednisolone IV followed by 2 mg/kg/day of prednisolone;

21th day

Persisting fever

Inflammatory markers still high;

2^nd administration of IVIG

29^th day

Fever decline; improvement of feet vasculitis and BCG scar inflammation

3^rd echocardiogram: increasing dilatation of left coronary artery; pericardial effusion.

Restarted methylprednisolone IV (30 mg/kg/day), followed by 2 mg/kg/day of prednisolone.

35^th day

Hospital discharge

Persisting abnormal levels of inflammatory markers;

Discharge prescription: ASA 5 mg/kg/day and clopidogrel 3.75 mg/kg/day

51^st day

Persisting abnormal levels of inflammatory markers; 4^th echocardiogram: aneurysms on right and left coronary arteries and dilatation of the descending branch of left coronary artery.

Methotrexate 0.5 mg/kg/week P.O. and etanercept 0.8 mg/kg/week added to drugs already in use

85^th day

Considered in clinical remission

Normal results of inflammatory activity tests; 5^th echocardiogram : reduction of aneurysm and anterior descending artery dilatation