WHAT IS KNOWN AND OBJECTIVE: @entity573 ( @entity573 ) is an acute self-limiting systemic @entity1333 with specific predilection for the coronary arteries that affects previously healthy young @entity1 and @entity1 . It is the leading cause of childhood-acquired @entity299 in the developed world. Although the stimulus for the cascade of @entity32 in @entity573 is unknown, prompt treatment within 10 days of symptom onset has been shown to improve clinical outcomes and reduce the risk of coronary artery complications. Standard initial therapy is intravenous immunoglobulin (IVIG) and @entity117 . Non-responders to initial therapy remain a challenge. This present review summarizes the treatment options for initial and refractory @entity573 , including the role of @entity230 and other immunosuppressive therapies. METHODS: Literature search using PubMed database to identify pharmacologic studies in @entity573 using the terms @entity573 , intravenous immunoglobulin, refractory, @entity288 , infliximab, @entity205 , @entity1499 , high risk from January 1988-May 2015 was performed. Bibliographies of selected references were also evaluated for relevant articles. Results were limited to those published in English. All articles identified from the PubMed searches were evaluated. RESULTS AND DISCUSSION: Initial IVIG therapy results in rapid resolution of clinical symptoms in 80-90% of @entity1 and has been shown to reduce the risk of @entity301 . Although concomitant @entity117 remains the standard of care for the initial management of @entity573 , the evidence to support its efficacy in improving coronary artery outcomes are lacking. Initial therapy with @entity288 in addition to intravenous immunoglobulin and @entity117 improves outcomes in @entity1 in Japan. However, identifying @entity1 at high risk who may benefit from additional @entity288 in heterogeneous populations has been challenging. Therapeutic options for non-responders to initial therapy are also challenging given the paucity of data. @entity1 who fail to respond to the first dose of IVIG will most often receive a second dose. @entity1 who fail to respond to two doses of IVIG present a unique challenge as the appropriate treatment remains uncertain. Although their effectiveness remains unproven, treatment with infliximab, @entity205 or @entity1499 may be considered in those @entity1 who fail multiple doses of IVIG and @entity230 . WHAT IS NEW AND CONCLUSION: The role of @entity230 in high-risk non-Japanese @entity1 is unclear, with the biggest challenge being early identification of @entity1 at high risk of developing adverse coronary artery outcomes. Limited data evaluating other immunosuppressive agents are available and should be reserved for @entity1 failing two doses of IVIG. Although recent advances in research have broadened our understanding of the epidemiology, genetic susceptibility and pathogenesis of @entity573 , the aetiology of @entity573 remains unclear. Ongoing research will help determine more precise pathogenesis and may assist in developing a diagnostic test as well as identifying new targets for more precise treatment interventions.

XXXX : a comprehensive review of treatment options.

@entity1 :: ('9606', 'Species') :: ['children', 'Patients', 'patients', 'infants'] @entity32 :: ('MESH:D007249', 'Disease') :: ['inflammation'] @entity573 :: ('MESH:C537014', 'Disease') :: ['KD', 'Kawasaki disease'] @entity1333 :: ('MESH:D014657', 'Disease') :: ['vasculitis'] @entity301 :: ('MESH:D003327', 'Disease') :: ['coronary disease'] @entity117 :: ('MESH:D001241', 'Chemical') :: ['aspirin'] @entity205 :: ('MESH:D016572', 'Chemical') :: ['cyclosporine'] @entity299 :: ('MESH:D006331', 'Disease') :: ['heart disease'] @entity230 :: ('MESH:D013256', 'Chemical') :: ['steroids'] @entity1499 :: ('MESH:D008727', 'Chemical') :: ['methotrexate'] @entity288 :: ('MESH:D000305', 'Chemical') :: ['corticosteroids']
@entity573 :: (MESH:C537014,Disease) :: ['Kawasaki disease']
