Kawasaki Disease Medication

Updated: Jul 29, 2018
  • Author: Tina K Sosa, MD; Chief Editor: Russell W Steele, MD  more...
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Medication

Immunomodulatory agents

Class Summary

IVIG is a purified preparation of gamma globulin. It is derived from large pools of human plasma comprising 4 subclasses of antibodies, approximating the distribution of human serum.

These agents are used to improve clinical and immunologic aspects of Kawasaki disease. They may decrease autoantibody production and increase solubilization and removal of immune complexes.

Immune globulin, intravenous (Carimune, Gammagard, Gamunex-C, Octagam)

IVIG is generally recommended as first-line therapy. It neutralizes circulating myelin antibodies by means of anti-idiotypic antibodies; downregulates proinflammatory cytokines, including interferon-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; and promotes remyelination. It may increase cerebrospinal fluid IgG levels (10%).

IVIG reduces the prevalence of coronary abnormalities. It leads to rapid defervescence and more rapid normalization of acute-phase reactants.

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Nonsteroidal Anti-Inflammatory Agents/Salicylates (NSAIDs)

Class Summary

These agents inhibit prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2. Adequate anti-inflammatory therapy requires that aspirin be combined with gamma globulin. Children with coronary artery disease have received aspirin for prolonged periods.

Aspirin (Ascriptin, Bayer Aspirin, Bayer Buffered Aspirin, Ecotrin)

Aspirin is used to decrease inflammation, inhibit platelet aggregation, and improve complications of venous stasis and thrombosis. It irreversibly inactivates cyclooxygenase, ultimately preventing thromboxane A2 production in platelets. Platelet function does not fully recover until new platelets are made (in 7-10 days). It is first-line therapy, along with IVIG.

Oral absorption may decrease in Kawasaki disease to < 50% (vs typical bioavailability of 85-90%). Altered bioavailability may explain why higher doses are required to achieve a salicylate serum concentration >20 mg/dL.

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Antiplatelet Agents, Hematologic

Class Summary

Besides aspirin, dipyridamole may be used to prevent microthrombus formation.

Dipyridamole (Persantine)

Dipyridamole is a platelet-adhesion inhibitor that possibly inhibits red blood cell uptake of adenosine, itself an inhibitor of platelet reactivity. It may inhibit phosphodiesterase activity, leading to increased cyclic adenosine monophosphate (cAMP) levels in platelets and formation of potent platelet activator thromboxane A2.

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Corticosteroids

Class Summary

Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

Prednisolone acetate (Prelone, Flo-Pred, Millipred)

Prednisolone is indicated for the treatment of steroid-responsive inflammation of the palpebral and bulbar conjunctiva, cornea, and anterior segment. It decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing increased capillary permeability.

Methylprednisolone (A-Methapred, Medrol, Solu-Medrol)

Methylprednisolone decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reversing increased capillary permeability.

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Immunosuppressants

Class Summary

These agents inhibit key factors that mediate immune reactions.

Infliximab (Remicade)

Infliximab may be added to treatment if steroids and other immunosuppressant drugs are ineffective in achieving or maintaining remission. Infliximab is a chimeric IgG1k monoclonal antibody that neutralizes cytokine TNF-α and inhibits its binding to the TNF-α receptor. It reduces the infiltration of inflammatory cells and TNF-α production in inflamed areas.

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Antiplatelet Agents, Cardiovascular

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