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Kawasaki Disease: Treatment & Medication
Updated: May 8, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
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Treatment
Medical Care
- The initial goals of therapy are to reduce the fever and the inflammation of the myocardium and to prevent subsequent cardiac sequelae. The current recommended therapy for KD in the acute phase includes a single infusion of intravenously administered gamma globulin and aspirin.15,22,23
- High-dose aspirin at a dose of 80-100 mg/kg/d orally in 4 equally divided doses is continued in the acute phase for its anti-inflammatory effects. It is continued at this dose until day 14 of the illness or until the patient has been afebrile for 48-72 hours.
- IVIG has a synergistic effect with aspirin and reduces acute inflammation, with the maximal benefits seen when it is given within the first 10 days of the illness. IVIG has been shown to reduce the rate of coronary aneurysms from greater than 25% in untreated patients to 1-5% in treated patients. In the past, IVIG was given as a lower dose over 4 days (400 mg/kg/d), but newer studies have shown that high single doses are more effective. In current practice, the dose is 2 g/kg intravenously over 10-12 hours.
- In patients in whom the standard treatment fails (approximately 10% of patients with KD) and who continue to remain febrile 36 hours after the initial dose of IVIG, a second treatment with IVIG at the original dose is recommended.24
- Of children, 10-20% are resistant to IVIG therapy and have the highest risk for coronary artery lesions. In the future, by identifying a genetic signature for this group, more aggressive therapies, such as anticytokine therapy, plasmapheresis, or cyclosporin A, may be used to reduce the risk of coronary complications.2
- Once the patient has remained afebrile for 48-72 hours, low-dose aspirin is initiated for its antiplatelet activity. The dose is 3-5 mg/kg/d for a total of 6-8 weeks as long as the patient shows no evidence of coronary abnormalities.
- For patients who have aneurysms, aspirin should be continued until the aneurysm resolves or should be continued indefinitely.
- Patients on prolonged aspirin therapy must be instructed that concomitant use of ibuprofen antagonizes the irreversible effect of platelet inhibition by aspirin and should be avoided during therapy.
- Patients who remain on long-term, low-dose aspirin should receive an annual influenza vaccine. Additionally, the risks of developing Reye syndrome during an active infection with influenza or varicella should be addressed.
Consultations
- Admit all patients to the hospital for administration of intravenous gamma globulin and for observation until fever is controlled. Closely monitor cardiovascular function.
- Consult with pediatric or adult infectious disease specialists to exclude infectious disease as a cause of fever.
- Consult with pediatric or adult cardiologists for the following:
- Children or adults with significant coronary artery disease
- To determine the timing of subsequent echocardiographic studies
- To initiate anticoagulation therapy in addition to aspirin in patients with large aneurysms
- To determine if other studies to assess cardiac function (eg, stress testing, coronary artery angiography) are required
Activity
Contact activities or high-impact sports should be avoided in patients on prolonged antiplatelet therapy.
Medication
The goals of pharmacotherapy are to reduce inflammation and platelet activation.
Immune globulins
These agents are used to improve clinical and immunologic aspects of the disease. They may decrease autoantibody production and increase solubilization and removal of immune complexes.
Intravenous immune globulin (Gammagard, Gamimune, Sandoglobulin, Gammar-P)
Neutralizes circulating antibodies and down-regulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells; augments suppressor T cells; blocks complement cascade.
Adult
2g/kg IV over 10-12 h
Gammagard S/D: 1 g/kg as single dose or 400 mg/kg for 4 consecutive days beginning within 7 d of fever onset; administration concomitantly with appropriate aspirin therapy (80-100 mg/kg/d divided qid) recommended
Pediatric
Administer as in adults
Antibodies in globulin preparation may interfere with response to live viral vaccines (eg, MMR); defer using live viral vaccines until approximately 11 mo after immunoglobulin administration; no known drug interactions
No absolute contraindication other than documented hypersensitivity; patients who are IgA deficient should receive IVIG preparations with no IgA; anti-IgE/IgG antibodies, severe thrombocytopenia, or coagulation disorders
Gammagard S/D contains only trace amounts of IgA and is not indicated in patients with selective IgA deficiency in whom the IgA deficiency is the only abnormality of concern, and it should be given with caution to patients with antibodies to IgA or IgA deficiencies that are a component of an underlying primary immunodeficiency disease for which IVIG therapy is indicated; in such instances, a risk of anaphylaxis may exist despite the fact that Gammagard S/D contains only trace amounts of IgA
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Check serum IgA level before IVIG (use an IgA-depleted product, eg, Gammagard S/D); infusions may increase serum viscosity and thromboembolic events; infusions may increase risk of migraine attacks, aseptic meningitis (10%), urticaria, pruritus, or petechiae (2-30 d postinfusion); increases risk of renal tubular necrosis in elderly patients and in patients with diabetes, volume depletion, and preexisting kidney disease; laboratory result changes associated with infusions include elevated antiviral or antibacterial antibody titers for 1 mo, 6-fold increase in ESR for 2-3 wk, and apparent hyponatremia
Nonsteroidal anti-inflammatory and antiplatelet agents
Inhibit prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2; act on heat-regulating center of hypothalamus and vasodilate peripheral vessels to reduce fever. By inhibiting prostaglandin synthesis, aspirin may also inhibit key steps in inflammation process.
Aspirin (Anacin, Ascriptin, Bayer Aspirin)
Aspirin is administered for its anti-inflammatory effects.
Adult
Acute phase: 80-100 mg/kg/d PO qid until patient remains afebrile for 48-72 h
Subacute phase: 3-5 mg/kg/d PO qd in morning, usually continued for 3 mo until ESR and platelet count return to normal
In Japan, clinicians generally use a lower antipyretic dose of 30-50 mg/kg/d
Pediatric
Administer as adults
Obtain aspirin levels if patient experiences hyperventilation, hyperpnea, diaphoresis, vomiting, tinnitus, liver function abnormalities or evidence of CNS depression develop; discontinue if evidence of Reye syndrome (eg, elevated LFT results, vomiting, alterations in CNS function, hyperactive reflexes, hyperventilation) develops or if signs of an allergic reaction or hypersensitivity reaction (eg, rash, toxic epidermal necrolysis, urticaria, angioedema, agranulocytosis, aplastic anemia, bronchospasm, elevated hepatic enzyme levels, erythema nodosum) occurs
Documented hypersensitivity; liver damage, hypoprothrombinemia, vitamin K deficiency, bleeding disorders, asthma; because of association with Reye syndrome, do not use in children (<16 y) with viral infections (eg, varicella, influenza)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May cause transient decrease in renal function and aggravate chronic kidney disease; avoid use in patients with severe anemia, peptic ulcer disease, a history of blood coagulation defects, or taking anticoagulants. Monitor salicylate level so that dose is maintained at 18-28 mg/dL
More on Kawasaki Disease |
| Overview: Kawasaki Disease |
| Differential Diagnoses & Workup: Kawasaki Disease |
 Treatment & Medication: Kawasaki Disease |
| Follow-up: Kawasaki Disease |
| Multimedia: Kawasaki Disease |
| References |
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References
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Further Reading
Keywords
mucocutaneous lymph node syndrome, Kawasaki's syndrome, Kawasaki's disease, Kawasaki syndrome, KD, infantile polyarteritis nodosa, IPAN, infantile periarteritis nodosa, medium-sized vessel vasculitis, medium vessel vasculitis, vasculitis of medium vessels, periarteritis nodosa
