Diagnostic Evaluation in Suspected Kawasaki Disease

1. Complete blood count and differential

2. Throat and nasal cultures

3. Nasopharyngeal swab for adenovirus, rapid direct fluorescent antigen test

4. Urinalysis

5. Erythrocyte sedimentation rate, C-reactive protein

6. Electrocardiogram

7. Echocardiogram or magnetic resonance angiography

8. BUN, creatinine, SGOT, SGPT

The principal differential diagnosis of Kawasaki syndrome includes scarlet fever, Staphylococcal scalded skin syndrome, toxic shock syndrome, and adenovirus infection. A summary of important features of each is given in Table 36.1.

Table 36.1. Differential Diagnosis of Kawasaki Disease

Kawasaki Disease

SS

Scarlet Fever

Toxic Shock Syndrome

Adenovirus (10)

Age group

>3 months, <5 years

<3 months most

2-10 years most

Menstruating

Usually <10 years

common, but

common

women,

any age

uncommon in

children

Conjunctival

+, injection

+, purulent

-

+/-, injection

Usually present

involvement

Strawberry

+

-

+, white early on

+/-

rare

tongue

Lip

+

-

-

-

-

involvement

Acral

+

+, but part of

-

+, but part of diffuse

-

diffuse involvement

involvement

Perineal

+

+, but part of

+/-

+/-

-

diffuse involvement

Bullae

-

+

-

-

-

Other

Cervical adenopathy,

May be able to culture

+throat culture,

+culture for

May have exudative

may have aseptic

Staphylococcus aureus

group A

Staphylococcus

pharyngitis and

meningitis, pyuria

Streptococcus,

aureus, sometimes

conjunctivitis

truncal exanthem

group A

accentuated in skin

Streptococcus,

folds, "sandpaper"

from primary site

texture on skin

of infection,

scarlatiniform

eruption,

rhabdomyolysis,

liver dysfunction,

thrombocytopenia

The typical pathologic finding in Kawasaki disease is that of a vasculitis, occurring most commonly in the coronary arteries, but also occurring in renal, iliac, femoral, and mesenteric arteries. There are initially subendothelial cell collections of macrophages, lymphocytes, and neutrophils (11). Plasma cells and eosinophils are also constituents of the infiltrate (5,12). The inflammation eventually becomes transmural, and can extend along the adventitia as in polyarteritis nodosa. In Kawasaki disease, in contrast to polyarteritis nodosa, there are fewer neutrophils, and fibrinoid necrosis is not prominent (11). Biopsy specimens of skin in Kawasaki disease do not have specific findings, but in those with sterile pustules, the location is subcor-neal (8).

The mainstay of treatment of Kawasaki disease is intravenous gammaglobulin, with aspirin. A comparison of treatment with aspirin alone versus aspirin with gamma-globulin reveals a striking reduction in the development of coronary aneurysms in the gammaglobulin group, and greater resolution of existing coronary lesions over time in the same group (13). Treatment with gammaglobulin is generally given using 2 mg/kg as a single infusion (3). High-dose aspirin is recommended early on in the disease to prevent thrombotic events, 80-100 mg/kg per day, divided in four doses, for up to 14 days, and then 3-5 mg/ kg as a single daily dose for seven weeks or longer (14). Some authors advocate lower doses of aspirin (15). Aspirin does not reduce aneurysm formation (16). A recent retrospective study has called into question the benefit of aspirin therapy, but for the present, it remains part of the standard treatment (17). Eighty-nine percent of patients respond to a single dose of gammaglobulin, but the remainder will remain febrile. A second dose of gamma-globulin has been advocated in those remaining febrile for the 48 to 72 hours following the first dose. Of these, two-thirds will become afebrile with the second dose (18). Patients with persistent fever after one dose of gamma-globulin, who receive additional gammaglobulin, have a decrease in cardiac complications (19). Treatment options for those refractory to two doses of intravenous gamma-globulin include additional doses of gammaglobulin, pulse methylprednisolone, infliximab, cyclosporine, cyclophosphamide, and plasmapheresis (3,20). A small trial of pulse methylprednisolone in addition to gamma-globulin and aspirin revealed more rapid resolution of symptoms in the group treated with steroids compared to the standard treatment group (gammaglobulin and aspirin), but no differences in cardiac outcome between the two groups (21).

The principal complication of Kawasaki disease is coronary artery disease. Sudden death occurs in 1%-2% of patients in the acute phase of the disease, and coronary aneurysms develop in 25% of patients with the disease; 55% eventually regress, but some are complicated by ste nosis or occlusion (22). Patients with giant aneurysms (those greater than 8 mm in diameter) are at considerably higher risk for complications (3). Lifelong follow-up is warranted in those with cardiac abnormalities. Recent data suggest that persistent coronary lesions tend to occur in patients with persistently elevated indices of inflammation, such as C-reactive protein, serum amyloid-A, inter-leukin-6, and soluble intercellular adhesion molecule-1 (23). This potential functional relationship may have implications for long-term treatment. Recurrent skin peeling has been described in patients with a history of Kawasaki disease, without any other evidence of disease reactivation. Some of these episodes have been associated with respiratory tract infections (24).

Kawasaki disease may resemble several bacterial toxin-mediated diseases, and, less likely, viral infections. Early diagnosis is important because of the ability to dramatically decrease the risk of complications with gammaglob-ulin treatment.

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