2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease

Mark Gorelik, Sharon A. Chung, Kaveh Ardalan, Bryce A. Binstadt, Kevin Friedman, Kristen Hayward, Lisa F. Imundo, Sivia K. Lapidus, Susan Kim, Mary Beth Son, Sangeeta Sule, Adriana H. Tremoulet, Heather Van Mater, Cagri Yildirim-Toruner, Carol A. Langford, Mehrdad Maz, Andy Abril, Gordon Guyatt, Amy M. Archer, Doyt L. ConnKathy A. Full, Peter C. Grayson, Maria F. Ibarra, Peter A. Merkel, Rennie L. Rhee, Philip Seo, John H. Stone, Robert P. Sundel, Omar I. Vitobaldi, Ann Warner, Kevin Byram, Anisha B. Dua, Nedaa Husainat, Karen E. James, Mohamad Kalot, Yih Chang Lin, Jason M. Springer, Marat Turgunbaev, Alexandra Villa-Forte, Amy S. Turner, Reem A. Mustafa

Research output: Contribution to journalArticlepeer-review

16 Scopus citations


Objective: To provide evidence-based recommendations and expert guidance for the management of Kawasaki disease (KD), focusing on clinical scenarios more commonly addressed by rheumatologists. Methods: Sixteen clinical questions regarding diagnostic testing, treatment, and management of KD were developed in the Patient/Population, Intervention, Comparison, and Outcomes (PICO) question format. Systematic literature reviews were conducted for each PICO question. We used the Grading of Recommendations, Assessment, Development and Evaluation method to assess the quality of evidence and formulate recommendations. Each recommendation required consensus from at least 70% of the Voting Panel. Results: We present 1 good practice statement, 11 recommendations, and 1 ungraded position statement to guide the management of KD and clinical scenarios of suspected KD. These recommendations for KD are focused on situations in which input from rheumatologists may be requested by other managing specialists, such as in cases of treatment-refractory, severe, or complicated KD. The good practice statement affirms that all patients with KD should receive initial treatment with intravenous immunoglobulin (IVIG). In addition, we developed 7 strong and 4 conditional recommendations for the management of KD or suspected KD. Strong recommendations include prompt treatment of incomplete KD, treatment with aspirin, and obtaining an echocardiogram in the setting of unexplained macrophage activation syndrome or shock. Conditional recommendations include use of IVIG with other adjuvant agents for patients with KD and high-risk features of IVIG resistance and/or coronary artery aneurysms. These recommendations endorse minimizing risk to the patient by using established therapy promptly at disease onset and identifying situations in which adjunctive therapy may be warranted. Conclusion: These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and use of echocardiography in patients with suspected or confirmed KD.

Original languageEnglish (US)
Pages (from-to)538-548
Number of pages11
JournalArthritis Care and Research
Issue number4
StatePublished - Apr 2022

Bibliographical note

Funding Information:
We thank Anne M. Ferris, MBBS, Ora Gewurz-Singer, MD, Rula Hajj-Ali, MD, Eric Matteson, MD, MPH, Robert F. Spiera, MD, Linda Wagner-Weiner, MD, MS, and Kenneth J. Warrington, MD, for serving on the Expert Panel. We thank Antoine G. Sreih, MD and Gary S. Hoffman, MD, MS, for their contributions during the early phases of this project as members of the Core Team. Dr. Hoffman's participation ended July 2018 due to personal reasons. Dr. Sreih's involvement ended in December 2018 when he became primarily employed by industry, which precluded his continued participation in this project. We thank Joyce Kullman (Vasculitis Foundation) for her assistance with recruitment for the Patient Panel. We thank the patients who (along with authors Kathy A. Full and Omar I. Vitobaldi) participated in the Patient Panel meeting: Jane Ascroft, Scott A. Brunton, Dedra DeMarco, Thomas Fitzpatrick, Jenn Gordon, Maria S. Mckay, Sandra Nye, Stephanie Sakson, and Ben Wilson. We thank Robin Arnold, Catherine E. Najem, MD, MSCE, and Amit Aakash Shah, MD, MPH, for their assistance with the literature review. We thank the ACR staff, including Ms. Regina Parker, for assistance in organizing the face-to-face meeting and coordinating the administrative aspects of the project, and Ms. Cindy Force for assistance in manuscript preparation. We thank Ms. Janet Waters for help in developing the literature search strategy and performing the initial literature search, and Ms. Janet Joyce for performing the update searches.

Funding Information:
Supported by the American College of Rheumatology and the Vasculitis Foundation.

Publisher Copyright:
© 2022 American College of Rheumatology. This article has been contributed to by US Government employees and their work is in the public domain in the USA.


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