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. Conn; Kathy 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

doi:10.1002/acr.24838

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

Pediatrics - Rheumatology Division

Research output: Contribution to journal › Article › peer-review

15 Scopus citations

Abstract

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 language	English (US)
Pages (from-to)	538-548
Number of pages	11
Journal	Arthritis Care and Research
Volume	74
Issue number	4
DOIs	https://doi.org/10.1002/acr.24838
State	Published - 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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Gorelik, M., Chung, S. A., Ardalan, K., Binstadt, B. A., Friedman, K., Hayward, K., Imundo, L. F., Lapidus, S. K., Kim, S., Son, M. B., Sule, S., Tremoulet, A. H., Van Mater, H., Yildirim-Toruner, C., Langford, C. A., Maz, M., Abril, A., Guyatt, G., Archer, A. M., ... Mustafa, R. A. (2022). 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease. Arthritis Care and Research, 74(4), 538-548. https://doi.org/10.1002/acr.24838

Gorelik, M, Chung, SA, Ardalan, K, Binstadt, BA, Friedman, K, Hayward, K, Imundo, LF, Lapidus, SK, Kim, S, Son, MB, Sule, S, Tremoulet, AH, Van Mater, H, Yildirim-Toruner, C, Langford, CA, Maz, M, Abril, A, Guyatt, G, Archer, AM, Conn, DL, Full, KA, Grayson, PC, Ibarra, MF, Merkel, PA, Rhee, RL, Seo, P, Stone, JH, Sundel, RP, Vitobaldi, OI, Warner, A, Byram, K, Dua, AB, Husainat, N, James, KE, Kalot, M, Lin, YC, Springer, JM, Turgunbaev, M, Villa-Forte, A, Turner, AS & Mustafa, RA 2022, '2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease', Arthritis Care and Research, vol. 74, no. 4, pp. 538-548. https://doi.org/10.1002/acr.24838

@article{fb61427c0b1a4d9c93825de5a3a2ad73,

title = "2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease",

abstract = "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.",

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

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: {\textcopyright} 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.",

year = "2022",

month = apr,

doi = "10.1002/acr.24838",

language = "English (US)",

volume = "74",

pages = "538--548",

journal = "Arthritis Care and Research",

issn = "2151-464X",

publisher = "John Wiley & Sons Inc.",

number = "4",

}

TY - JOUR

T1 - 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease

AU - Gorelik, Mark

AU - Chung, Sharon A.

AU - Ardalan, Kaveh

AU - Binstadt, Bryce A.

AU - Friedman, Kevin

AU - Hayward, Kristen

AU - Imundo, Lisa F.

AU - Lapidus, Sivia K.

AU - Kim, Susan

AU - Son, Mary Beth

AU - Sule, Sangeeta

AU - Tremoulet, Adriana H.

AU - Van Mater, Heather

AU - Yildirim-Toruner, Cagri

AU - Langford, Carol A.

AU - Maz, Mehrdad

AU - Abril, Andy

AU - Guyatt, Gordon

AU - Archer, Amy M.

AU - Conn, Doyt L.

AU - Full, Kathy A.

AU - Grayson, Peter C.

AU - Ibarra, Maria F.

AU - Merkel, Peter A.

AU - Rhee, Rennie L.

AU - Seo, Philip

AU - Stone, John H.

AU - Sundel, Robert P.

AU - Vitobaldi, Omar I.

AU - Warner, Ann

AU - Byram, Kevin

AU - Dua, Anisha B.

AU - Husainat, Nedaa

AU - James, Karen E.

AU - Kalot, Mohamad

AU - Lin, Yih Chang

AU - Springer, Jason M.

AU - Turgunbaev, Marat

AU - Villa-Forte, Alexandra

AU - Turner, Amy S.

AU - Mustafa, Reem A.

N1 - 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.

PY - 2022/4

Y1 - 2022/4

N2 - 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.

AB - 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.

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

Abstract

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