Infliximab treatment for refractory Kawasaki syndrome

• Published in: The Journal of pediatrics Vol. 146; no. 5; pp. 662 - 667
• Main Authors: Burns, Jane C., Mason, Wilbert H., Hauger, Sarmistha B., Janai, Hillel, Bastian, John F., Wohrley, Julie D., Balfour, Ian, Shen, Cynthia A., Michel, Edward D., Shulman, Stanford T., Melish, Marian E.
• Format: Journal Article
• Language: English
• Published: New York, NY Mosby, Inc 01.05.2005; Elsevier
• Subjects: Adolescent; Anti-Inflammatory Agents, Non-Steroidal - therapeutic use; Antibodies, Monoclonal - therapeutic use; Antirheumatic Agents - therapeutic use; Aspirin - therapeutic use; Biological and medical sciences; Child; Child, Preschool; Female; Fever - drug therapy; General aspects; Humans; Immunoglobulins, Intravenous; Infant; Infliximab; Male; Medical sciences; Mucocutaneous Lymph Node Syndrome - drug therapy; Mucocutaneous Lymph Node Syndrome - physiopathology; Retrospective Studies; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Treatment Outcome; Tumor Necrosis Factor-alpha - antagonists & inhibitors; CRP; CAA; eNOS; KS; IVIG; TNF; Ig; ASA; Kawasaki syndrome; Pediatrics; Treatment resistance; Cardiovascular disease; Autoimmune disease; Monoclonal antibody; Vascular disease; Immunomodulator; Refractory; Vasculitis; Treatment; Infliximab; Anticytokine; Systemic disease; Immunosuppressive agent
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/j.jpeds.2004.12.022

To evaluate the use of tumor necrosis factor (TNF)-α blockade for treatment of patients with Kawasaki syndrome (KS) who fail to become afebrile or who experience persistent arthritis after treatment with intravenous gamma globulin (IVIG) and high-dose aspirin. Cases were retrospectively collected from clinicians throughout the United States who had used infliximab, a chimeric murine/human immunoglobulin (Ig)G1 monoclonal antibody that binds specifically to human TNF-α-1, for patients with KS who had either persistent arthritis or persistent or recrudescent fever ≥48 hours following infusion of 2 g/kg of IVIG. Response to therapy with cessation of fever occurred in 13 of 16 patients. C-reactive protein (CRP) level was elevated in all but one patient before infliximab infusion, and the level was lower following infusion in all 10 patients in whom it was re-measured within 48 hours of treatment. There were no infusion reactions to infliximab and no complications attributed to infliximab administration in any of the patients. The success of TNF-α blockade in this small series of patients suggests a central role of TNF-α in KS pathogenesis. Controlled, randomized clinical trials are warranted to determine the role of anti-TNF-α therapy in KS.