CAUSES
. Unknown
. Microbial agent is favored because of acute, self-limited course and community-wide outbreaks
. Leading theory is that a staphylococcal or streptococcal superantigen, in the appropriate host, stimulates T cell populations which in turn cause activation of immune responses directed against endothelial cell antigens
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DIFFERENTIAL DIAGNOSIS
β’ Staphylococcal scalded skin syndrome
β’ Toxic shock syndrome
β’ Stevens-Johnson syndrome
β’ Reiter syndrome
β’ Juvenile rheumatoid arthritis
β’ Scarlet fever
β’ Measles
β’ Rubella
β’ Roseola
β’ Epstein-Barr virus infections
β’ Mycoplasma infection
β’ Leptospirosis
β’ Lyme disease
β’ Rocky Mountain spotted fever
β’ Toxoplasmosis
β’ Acrodynia
β’ Drug reactions
β’ Other vasculitides
LABORATORY
β’ Anemia (normochromic, normocytic)
β’ Leukocytosis (12,000-40,000 cells/mm3) with immature forms
β’ Elevated CRP, ESR, and alpha1-antitrypsin concentrations
β’ Platelet counts rise in subacute phase and peak in convalescent at 750,000-1,500,000
β’ Thrombocytopenia associated with severe coronary disease and myocardial infarction
β’ Mildly elevated serum liver enzymes and bilirubin
β’ CSF pleocytosis may be seen
β’ Measles immunoglobulin (IgM) titer helpful in differentiating KS from measles
β’ Sterile urethral pyuria occurs in 30% of patients
SPECIAL TESTS
. Quantitative serum immunoglobulins
. ANA, RF, VDRL, immune complexes, complement levels
IMAGING
. Electrocardiogram may show ischemia, arrhythmias
. Echocardiogram may show cardiomyopathy, pericardial effusion, coronary artery dilatation or aneurysms
. Radiography including angiography
DIAGNOSTIC PROCEDURES
. No laboratory study proves diagnosis; diagnosis rests on clinical features and exclusion of other illnesses in differential diagnosis
. Diagnosis of typical syndrome requires fever of at least 5 days duration, plus 4 of the following 5 criteria:
. Mucous membrane changes
. Extremity changes
. Cervical lymphadenopathy of at least 1 cm in size
. Rash
. Conjunctival suffusion
. Atypical cases occur with incomplete clinical findings; frequency of coronary artery aneurysms may be higher in this subset of patients Kawasaki syndrome
* ACTIVATED HELPER-T CELLS & MONOCYTES ARE INCREASED IN NUMBER
* SERUM SOLUBLE INTERLEUKIN-2 RECEPTOR LEVELS - ELEVATED
* SPONTANEOUS INTERLEUKIN-1 LEVELS - ELEVATED
* PLATELET COUNT - INCREASED
* ANTIENDOTHELIAL CELL ANTIBODIES - PRESENT