Medication Summary
Current standard of care for Kawasaki disease (KD) includes slow intravenous immune globulin (IVIG). When considering this treatment, risks must be compared with benefits in atypical or questionable cases, since IVIG is a human blood product and possesses potential infectious hazards. Additionally, a nonsteroidal anti-inflammatory drug (NSAID) or aspirin is administered orally. The author prefers one of the NSAIDs, such as naproxen in anti-inflammatory doses or ibuprofen in antipyretic doses, because of their safety profiles. Low-dose aspirin may be continued for months for its antiplatelet effects. Dipyridamole may be prescribed for patients with coronary artery aneurysms.
The treatment of children with polyarteritis nodosa (PAN) must be individualized. Often, corticosteroids are required to reduce inflammation. High-dose IV pulse methylprednisolone is administered judiciously over 1 hour with appropriate monitoring (blood pressure q15min) to treat organ- or life-threatening disease. This treatment may be repeated daily for 3-6 days or on alternate days depending on the patient's response. Aggressive treatment with cytotoxic drugs must be individualized and may be lifesaving. Plasma exchange therapy, with or without hemodialysis, may be required. Because of the high fatality rate, a pediatric rheumatologist experienced in caring for the critically ill child with necrotizing vasculitis must direct and coordinate care.
Biologic agents have been used in the treatment of patients with severe and/or refractory PAN. [1, 9, 10]
Immune Globulin
Class Summary
Immune globulin is purified preparation of gamma globulin derived from large pools of human plasma and composed of 4 subclasses of antibodies, approximating the distribution of human serum. One blood product, immunoglobulin, has documented efficacy in childhood polyarteritis nodosa (CPAN).
Immunoglobulin, intravenous (Gamimune N, Gammagard, Sandoglobulin)
Neutralizes circulating myelin antibodies through anti-idiotypic antibodies. Also down-regulates proinflammatory cytokines, including INF-γ, blocks Fc receptors on macrophages, suppresses inducer T and B cells, and augments suppressor T cells. Blocks complement cascade and promotes remyelination. May increase CSF IgG (10%).
In controlled studies, IVIG rapidly decreases fever, shortens hospitalization, and decreases coronary aneurysms in patients with KD.
Nonsteroidal anti-inflammatory drugs
Class Summary
These agents have anti-inflammatory, antipyretic, and antiplatelet activities. Their mechanism of action inhibits cyclooxygenase activity and prostaglandin synthesis. Other mechanisms also may exist, such as inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell-membrane functions.
Naproxen (Naprosyn)
Anti-inflammatory, antipyretic, and antiplatelet activities all therapeutic in this disease. Because of relatively long serum half-life, can be used twice daily.
Ibuprofen (Motrin, Advil)
Excellent antipyretic activity. Because of short plasma half-life, must be administered qid.
Corticosteroids
Class Summary
These agents have potent anti-inflammatory effects. Intermittent high doses (ie, pulse therapy) of intravenous methylprednisolone inhibit antibody production.
Prednisone (Deltasone, Orasone, Liquid Pred)
Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocyte and antibody production. Effective in reducing severe inflammation in vasculitis.
Methylprednisolone (Solu-Medrol)
Decreases inflammation by suppressing migration of PMNs and reversing increased capillary permeability. High-dose pulse IV methylprednisolone has been lifesaving in patients with systemic vasculitis.
Immunosuppressive agents
Class Summary
Patients with immune dysregulation and autoimmunity often benefit from immunosuppression. One immunosuppression agent, cyclophosphamide, may be lifesaving in patients with severe vasculitis.
Cyclophosphamide (Cytoxan)
Has probably best risk-to-benefit ratio in systemic vasculitis of all antineoplastic agents.
-
Childhood polyarteritis nodosa. Bilateral axillary aneurysms.
-
Childhood polyarteritis nodosa. Gangrene of the forearm and lower leg in a 3-month-old infant.
-
Childhood polyarteritis nodosa. Multiple aneurysms on arteriogram and autopsy specimen.
-
Thrombosed cystic artery in childhood polyarteritis nodosa.
-
Childhood polyarteritis nodosa. Heart with 5 coronary aneurysms.
-
A: Right coronary artery stenosis. B: Stent in place across stenotic segment.
-
Close-up of stent in place.
