General Information
• It is a vasculitis involving the coronary arteries, previously called mucocutaneous lymph node syndrome or infantile PAN.
• It is the second most common childhood vasculitis after Henoch-Schönlein purpura.
• It often occurs under the age of 5 years. The average age at diagnosis is 3 years.
• Fever is the most common and first finding.
A. Fever lasting at least 5 days
B. At least four of the following:
1) Bilateral nonpurulent conjunctival congestion
2) Changes in the oropharyngeal mucosa: diffuse redness of the oropharynx, dried and chapped lips, strawberry tongue
3) Peripheral limb changes; Edema and/or erythema of hands and feet, usually periungual desquamation starts in the 2nd-3rd week.
4) Polymorphic but non-vesicular rash, usually on trunk
5) Unilateral cervical LAP greater than 1.5 cm in diameter
C. Disease that does not fit into other disease tables
Fever + <4 criteria (atypical or incomplete Kawasaki disease) diagnosis is made if coronary artery aneurysm is shown.
Clinic:
acute period:
• It is the first 1-2 week period. In addition to the above findings, myocarditis, transient arthritis and sterile pyuria are valuable findings for this period.
• Myocarditis is the most important cause of death in this period.
subacute period
• It is a period of 2-4 weeks.
• Coronary aneurysm and thrombosis, peeling, thrombocytosis, gallbladder hydrops are present.
recovery period
• It is a period of 6-8 weeks.
• It begins after all clinical signs have disappeared, and lasts until the ESR returns to normal.
Lab
• Leukocytosis, neutrophilia, left shift, thrombocytosis after the 1st week
• Normochromic normocytic anemia
• Increase in CRP and sedimentation
• ALT and AST increase
• Hyponatremia, hypoalbuminemia
• Sterile pyuria, leukocytosis in synovial fluid, pleocytosis in CSF
• ANA and RF are negative
Treatment
1. Acute period: IVIG + aspirin (80-100 mg/kg/day) until 48 hours after fever subsides.
2. Convalescent period: Aspirin (3-5 mg/kg/day) 6-8 weeks
3. Long-term therapy in the presence of coronary abnormalities: Aspirin ± Clopidogrel ± Warfarin or low molecular weight heparin
4. Acute coronary thrombosis: Fibrinolytic therapy (streptokinase, urokinase)
Methylprednisolone + IVIG, infliximab, cyclosporine, anakinra, cyclophosphamide, plasma exchange can be used in IVIG unresponsiveness in acute treatment.
Prognosis
• The most important complication of Kawasaki disease is coronary artery disease and acute myocardial infarction.
• When looking at all periods, the most common cause of death is coronary ischemia and acute myocardial infarction, while myocarditis is the most common cause of death in the acute period.
• The disease may recur in 1-3% of cases.
Harada and his freinds have defined some criteria to predict coronary artery involvement:
1. Male gender
2. Be less than 1 year old
3. White sphere>l2.000/mm3
4. Platelet count <350.000/mm3
5. Hematocrit < 35%
6. Albumin < 3.5 g/dl
7. CRP>3+
It has been suggested that in the presence of four, the risk of developing coronary artery aneurysm is high.
Differential Diagnosis in Kawasaki Syndrome:
• Bacteria: Scarlet fever (most confounding disease), bacterial cervical lymphadenitis, rickettsial infections, leptospirosis, meningococcemia
• Viruses: EBV, HHV6, Measles, Rubella, CMV, Adenovirus
• Rheumatologic: Systemic JIA, Behçet
• Toxins: Mercury
• Other: Drug hypersensitivity reactions, Stevens Johnson syndrome, toxic shock syndrome, staphylococcal scalded skin syndrome
