[Intravenous immunoglobulin-resistant Kawasaki disease with hemophagocytosis].
Kawasaki disease is acute self-limited vasculitis of unknown etiology that mainly affects infants and young children. Many different clinical aspects can be encountered. A single dose of intravenous immunoglobulin and treatment by aspirin are the standard therapy. Cases of immunoglobulin therapy resistance pose a real problem. We report on the case of a 14-year-old boy with Kawasaki disease and hemophagocytic syndrome, resistant to the combination of two doses of immunoglobulins and three doses of corticosteroids. Recovery was obtained with one dose of infliximab. This observation highlights Kawasaki disease in adolescents and the therapeutic difficulties that may be encountered in cases of resistance to immunoglobulins. Association with macrophage activation syndrome is rare.
Service de pédiatrie générale, maladies infectieuses, immunologie clinique, hôpital Arnaud-de-Villeneuve, centre hospitalier régional universitaire de Montpellier, 371, avenue du Doyen-Gaston-Giraud, 34295 Montpellier cedex 5, France.
A-C
AC
Servel
M
M
Vincenti
J-P
JP
Darras
M
M
Lalande
M
M
Rodière
A
A
Filleron
D000293:Adolescent; D004351:Drug Resistance; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications*; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications* / Q000188:drug therapy*
D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors