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

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 lea...

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Published inACR Open Rheumatology Vol. 3; no. 10; pp. 671 - 683
Main Authors James, Karen E., Kalot, Mohamad A., Husainat, Nedaa M., Dua, Anisha B., Byram, Kevin, Springer, Jason M., Lin, Yih Chang, Turgunbaev, Marat, Villa‐Forte, Alexandra, Gorelik, Mark, Abril, Andy, Langford, Carol, Maz, Mehrdad, Chung, Sharon A., Mustafa, Reem A.
Format Journal Article
LanguageEnglish
Published Atlanta Wiley 01.10.2021
John Wiley & Sons, Inc
John Wiley and Sons Inc
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Online AccessGet full text
ISSN2578-5745
2578-5745
DOI10.1002/acr2.11308

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Summary: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 ed 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.
Bibliography:Kevin Byram, MD, Jason M. Springer, MD, MS: Vanderbilt University Medical Center, Nashville, Tennessee
Mehrdad Maz, MD, Reem A. Mustafa, MD, MPH, PhD: University of Kansas Medical Center, Kansas City, KS
Alexandra Villa‐Forte, MD, MPH, Carol Langford, MD: Cleveland Clinic, Cleveland, Ohio
Yih Chang Lin, MD: University of South Florida, Tampa, Florida
Marat Turgunbaev, MD, MPH: Clinical Practice Guidelines, American College of Rheumatology, Atlanta, Georgia
Mark Gorelik, MD: Mayo Clinic, Jacksonville, Florida
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Mohamad A. Kalot, MD: State University of New York at Buffalo, Buffalo, New York
Anisha B. Dua, MD, MPH: Northwestern University Feinberg School of Medicine, Chicago, Illinois
This systematic review was conducted to support the development of the American College of Rheumatology 2020 guidelines for diagnosis and management of vasculitis. The entire guideline development process was funded by the American College of Rheumatology. Through the Outcomes and Implementation Research Unit at Kansas University Medical Center, some researchers received salary or grant support; others volunteered their time.
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Sharon A. Chung, MD, MAS: University of California, San Francisco Medical Center.
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No potential conflicts of interest relevant to this article were reported.
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Drs. James and Kalot contributed equally to this manuscript.
Nedaa M. Husainat, MD: St. Mary's Hospital, Saint Louis, Missouri
Karen E. James, MD, MSCE: University of Utah Health, Salt Lake City, Utah
Andy Abril, MD: Columbia University Irving Medical Center, New York, New York
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ISSN:2578-5745
2578-5745
DOI:10.1002/acr2.11308