Kawasaki Disease: A Systematic Review and Meta-Analysis of Benefits and Harms of Common Treatments

Karen E. James, Mohamad A. Kalot, Nedaa M. Husainat, Anisha B. Dua, Kevin Byram, Jason M. Springer, Yih Chang Lin, Marat Turgunbaev, Alexandra Villa-Forte, Mark Gorelik, Andy Abril, Carol Langford, Mehrdad Maz, Sharon A. Chung, Reem A. Mustafa*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

2 Scopus citations


Objective: Kawasaki disease (KD) is a self-limited vasculitis affecting medium-sized vessels with a predilection for the coronary arteries. Although treatment reduces the likelihood of developing of coronary artery aneurysms, 5% of patients still develop aneurysms despite treatment, making KD the leading cause of acquired heart disease in children in the United States. Consequently, there is a great deal of interest in optimizing treatment regimens, particularly for higher-risk patients, to decrease morbidity. The aim of this systematic review is to support the development of the American College of Rheumatology/Vasculitis Foundation for the diagnosis and management of KD, focusing on the more complex scenarios in which rheumatologists may become involved, such as high-risk and refractory disease. Methods: Eighty-nine articles were considered for full review in this systematic literature review to address 16 Population, Intervention, Comparison, and Outcome questions related to KD. Data were abstracted in hierarchical fashion. Randomized control trials (RCTs) were considered first; if none were identified or if they contained insufficient information, comparative observational studies were then viewed, followed by single-arm observational studies/single arms from comparative studies. Only observational studies with more than 10 subjects with vasculitis were included. Results: Eight RCTs and 28 observational studies that addressed the questions were identified. Two questions were addressed by RCTs, seven questions had at least some comparative observational studies, three questions were only addressed by single-arm data, and four questions had no relevant studies. Conclusion: This systematic review evaluates the benefits and harms of treatments for KD beyond first-line therapy.

Original languageEnglish (US)
Pages (from-to)671-683
Number of pages13
JournalACR open rheumatology
Issue number10
StatePublished - Oct 2021

ASJC Scopus subject areas

  • Rheumatology


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