Infliximab treatment for refractory Kawasaki syndrome.
OBJECTIVE
To evaluate the use of tumor necrosis factor (TNF)-alpha 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.


METHODS
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-alpha-1, for patients with KS who had either persistent arthritis or persistent or recrudescent fever > or =48 hours following infusion of 2 g/kg of IVIG.


RESULTS
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.


CONCLUSIONS
The success of TNF-alpha blockade in this small series of patients suggests a central role of TNF-alpha in KS pathogenesis. Controlled, randomized clinical trials are warranted to determine the role of anti-TNF-alpha therapy in KS.
Burns
Jane C
JC
Children's Hospital of San Diego, and Department of Pediatrics, UCSD School of Medicine, San Diego, CA, USA.
Mason
Wilbert H
WH
Hauger
Sarmistha B
SB
Janai
Hillel
H
Bastian
John F
JF
Wohrley
Julie D
JD
Balfour
Ian
I
Shen
Cynthia A
CA
Michel
Edward D
ED
Shulman
Stanford T
ST
Melish
Marian E
ME
D000293:Adolescent; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D001241:Aspirin / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005334:Fever / Q000188:drug therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous; D007223:Infant; D000069285:Infliximab; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000188:drug therapy* / Q000503:physiopathology; D012189:Retrospective Studies; D016896:Treatment Outcome; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*
D000894:Anti-Inflammatory Agents, Non-Steroidal; D000911:Antibodies, Monoclonal; D018501:Antirheumatic Agents; D016756:Immunoglobulins, Intravenous; D014409:Tumor Necrosis Factor-alpha; D000069285:Infliximab; D001241:Aspirin