Kawasaki Disease: an Update
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VASCULITIS (L ESPINOZA, SECTION EDITOR)
Kawasaki Disease: an Update Eileen Rife 1 & Abraham Gedalia 2
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review Provide the most recent updates on the epidemiology, pathogenesis, and treatment advances in Kawasaki disease. Recent Findings Treatment advances in complex, IVIG-refractory cases of Kawasaki disease. Multisystem inflammatory syndrome, a newly reported inflammatory condition with Kawasaki-like features and an association with the 2019 Coronavirus (COVID-19). Summary Kawasaki disease (KD) is a rare systemic inflammatory disease that predominately affects children less than 5 years of age. Pathogenesis of KD remains unknown; the leading theory is that an unknown stimulus triggers an immune-mediated inflammatory cascade in a genetically susceptible child. Classic KD is a clinical diagnosis based on set criteria and excluding other similar clinical entities. Patients who do not fulfill complete diagnostic criteria for KD are often referred to as atypical (or incomplete) KD. The most feared complication of KD is coronary artery abnormality development, and patients with atypical KD are also at risk. Administration of intravenous immunoglobulin (IVIG) and aspirin has greatly reduced the incidence of coronary lesions in affected children. Several other immune-modulating therapies have recently been utilized in complex or refractory cases. Keywords Kawasaki disease . Kawasaki review . Kawasaki treatment . Kawasaki workup . Kawasaki differential . Kawasaki diagnosis . Multi system inflammatory syndrome . Kawasaki-like disease
Introduction Kawasaki disease (KD) was first described in a 1967 report by Japanese pediatrician Tomisaku Kawasaki. The cardiac sequelae were later documented in 1970, following investigation of 10 autopsy cases of sudden cardiac death following diagnosis of KD. The first reported cases outside Japan were in Hawaii in the early 1970s; KD cases have since been reported in more than 60 countries worldwide.
Epidemiology The epidemiology of KD varies greatly by geographic location and seasonality. The highest incidence rates (per 100,000) This article is part of the Topical Collection on Vasculitis * Eileen Rife [email protected]
are in children of Japanese ancestry. Recently published data from the Japanese KD nationwide survey reported an increased rate over time from 218.6 per 100,000 in 2008 to 243.1 and 330.2 in 2011 and 2015 respectively [1•, 2]. In the United States, the incidence appears to have remained relatively stable. In 2012, the KD-associated hospitalization rate for children < 5 years of age was 18.1 per 100,000. In 2003, the rate was 19.7 per 100,000 children, [3] which amounts to roughly 4000 to 5500 new cases in the United States each year. The highest rates are seen among children < 5 years of age, with a male predominance (21.0 per 100,000 versus 15 per 100,000 in females). There is considerable ethnic variation, the highest rates seen among Asian/Pacific Islanders at 29.8 per 100,000 c
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