Abstract:

Kawasaki disease is a systemic vasculitis. Macrophage activation syndrome is a severe complication of pediatric rheumatic immune diseases, which is characterized by cytokine storms and has a rapid progression and high mortality rate. Early identification of Kawasaki disease-associated macrophage activation syndrome is difficult, and its main features include persistent fever, splenomegaly, elevated ferritin levels, and decreased platelets. At present, there is no uniform diagnostic criteria for this disease, which mainly refers to the diagnostic criteria of systemic juvenile idiopathic arthritis with macrophage activation syndrome and primary hemophagocytic lymphohistiocytosis. Meanwhile, this disease should be distinguished from Kawasaki disease shock syndrome and childhood multisystem inflammatory syndrome. Glucocorticoids are the main treatment drugs, and patients with poor response can use traditional immunosuppressants such as cyclosporine. Biologic agents and targeted drugs have broad prospects.

Key words: Kawasaki disease, macrophage activation syndrome, cytokine storm, child