IL-1 Inhibition May Have an Important Role in Treating Refractory Kawasaki Disease.

ABSTRACT: Kawasaki disease (KD) is an acute inflammatory vasculitis occurring in young children before 5 years and representing at this age, the main cause of acquired heart disease. A single infusion of 2 g/kg of intravenous immunoglobulins along with aspirin has reduced the frequency of coronary artery aneurysms from 25 to 5%. However, 10-20% of patients do not respond to standard treatment and have an increased risk of cardiac complications and death. The development of more potent therapeutic approaches of KD is an urgent need. Phenotypical and immunological similarities between KD and systemic juvenile idiopathic arthritis led to the hypothesis that KD could be considered as an autoinflammatory disease. New insights regarding KD's pathogenesis have merged from the combination of genetic and tra

SUBMITTER: Dusser P 

PROVIDER: S-EPMC5368266 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature