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IVIG in KD. A persistent fever may be a reaction to treatment with IVIG and therefore
children are not usually retreated until at least 36 hours after the initial IVIG infusion.
It is, however, extremely important to confirm the diagnosis; it must be remembered
that failure to respond to IVIG might indicate that the child has a different source of
fever, such as a bacterial or viral infection, or a chronic inflammatory disease.
Approximately two-thirds of children with KD who fail to respond to an initial dose
of IVIG improve with a second course. A small number seem to be resistant to IVIG,
and they should be treated with intravenous pulsed-dose methylprednisolone (30
mg/kg/day) for 3 days. Infliximab may be an alternative to a second infusion of IVIG
or corticosteroids for IVIG-resistant patients. Patients who fail to respond to this
regimen may be candidates for cyclosporine, cytotoxic agents, or other monoclonal
antibody therapy.

LYME DISEASE
Please see Chapter 94 Infectious Disease Emergencies .
Suggested Readings and Key References
Juvenile Rheumatoid Arthritis
Adams A, Lehman TJ. Update on the pathogenesis and treatment of systemic onset
juvenile rheumatoid arthritis. Curr Opin Rheumatol 2005;17:612–616.
Cassidy JT, Petty RE. Juvenile rheumatoid arthritis. In: Textbook of Pediatric
Rheumatology. 3rd ed. Philadelphia, PA: WB Saunders; 1995.
Cunnane G, Doran M, Bresnihan B. Infections and biological therapy in rheumatoid
arthritis. Best Pract Res Clin Rheumatol 2003;17:345–363.
Duffy CM, Colbert RA, Laxer RM, et al. Nomenclature and classification in chronic
childhood arthritis. Time for a change? Arthritis Rheum 2005;52:382–385.
Hashkes PJ, Laxer RM. Update on the medical treatment of juvenile idiopathic
arthritis. Curr Rheumatol Rep 2006;8:450–458.
Ilowite NT. Current treatment of juvenile rheumatoid arthritis. Pediatrics
2002;109:109–115.
Ilowite NT. Update on biologics in juvenile idiopathic arthritis. Curr Opin Rheumatol
2008;20:613–618.


Kelly A, Ramanan AV. Recognition and management of macrophage activation
syndrome in juvenile arthritis. Curr Opin Rheumatol 2007;19:477–481.
Murray K, Thompson SD, Glass DN. Pathogenesis of juvenile chronic arthritis: genetic
and environmental factors. Arch Dis Child 1997;77:530–534.
Schneider R, Passo MH. Juvenile rheumatoid arthritis. Rheum Dis Clin North Am
2002;28:503–530.
Systemic Lupus Erythematosus



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