The American College of Rheumatology (ACR) and Vasculitis Foundation released rheumatology-based guidelines for the management of Kawasaki disease (KD). The full report was published in Arthritis Care & Research.1

The authors noted that the guidelines were developed to supplement the comprehensive recommendations issued by the American Heart Association (AHA) in 2017 and address situations in which rheumatologists are frequently consulted.2

The current guidelines were developed using standard ACR methodology. A total of 16 clinical questions were formulated using the patient, intervention, comparison, and outcomes (PICO) framework. Literature reviews were conducted for each question. A voting panel assessed the quality of evidence in the literature and formulated recommendations based on the grading of recommendations, assessment, development, and evaluation (GRADE) method. A consensus of 70% of the voting panel was required to adopt a recommendation.


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The final guidelines included 1 good practice statement, 11 recommendations, and 1 ungraded position statement.

Recommendations were graded as strong or conditional. Strong recommendations were supported by high-quality evidence and apply to the majority of patients. Conditional recommendations were based on lower quality evidence; treating physicians may consider alternatives and use a shared decision-making approach while implementing these recommendations.

Overall, the guidelines emphasize the use of intravenous immunoglobulin (IVIG) as the standard-of-care for initial KD treatment, the importance of prompt KD diagnosis and treatment, and identifying conditions for the use of adjunctive therapy.

Good Practice Statement

  • The standard-of-care for the initial treatment of KD is IVIG.

Treatment Recommendations

  • The use of IVIG with adjunctive agents as initial therapy is conditionally recommended over treatment with IVIG alone for patients with acute KD who are at high risk for IVIG resistance or developing coronary artery aneurysms (CAAs). Adjunctive therapies include glucocorticoids and nonglucocorticoid immunomodulatory immunosuppressive agents. Treatment with IVIG alone may be considered if the patient’s risk category is unknown or if adjunctive therapy is inappropriate. Treating physicians are also encouraged to consult a rheumatologist before using adjunctive therapy.
  • For patients with incomplete KD (ie, prolonged, unexplained fever but that does not meet sufficient criteria for KD diagnosis), prompt treatment with IVIG is strongly recommended. Treating physicians should evaluate patients with incomplete KD using the AHA guidelines2 and initiate treatment as soon as an incomplete diagnosis is made rather than wait until day 10 to see if patients meet the criteria for complete KD.
  • Separate treatment for both KD and macrophage activation syndrome (MAS) is strongly recommended for patients with acute KD and suspected or diagnosed MAS. Inadequate treatment for either condition can cause severe complications.
  • For patients with acute KD and fever that lasts longer than 36 hours after initial treatment with IVIG, a second course of IVIG is conditionally recommended over glucocorticoids. However, glucocorticoids are a reasonable alternative, especially in patients with risk factors for IVIG-associated hemolytic anemia, as the current evidence does not clearly indicate the superiority of IVIG over glucocorticoids.
  • Aspirin is strongly recommended and considered the standard-of-care for its antiplatelet effects.
  • For patients with acute KD with resolution of fever, continued daily monitoring for fever for 1 to 2 weeks is strongly recommended, as fever is a predictor of CAAs.
  • For patients with acute KD with arthritis that continues after IVIG treatment and those who do not have CAAs, nonsteroid anti-inflammatory drug (NSAID) therapy is conditionally recommended. In these patients, aspirin can be suspended to allow for a 3- to 4-week course of NSAIDs.

Diagnostic Imaging Recommendations

  • An echocardiography (ECG) with coronary artery measurements as soon as possible is strongly recommended to confirm diagnosis for patients with incomplete KD.
  • For patients with unexplained shock physiology or unexplained MAS, an ECG with coronary artery measurements is strongly recommended to evaluate whether KD is the potential cause.

Ungraded Position Statement

  • Use of another agent (nonglucocorticoid immunosuppressive therapy or glucocorticoids) is recommended for patients with acute KD and persistent fevers after 2 doses with IVIG.

The authors of the guidelines concluded, “The ACR and Vasculitis Foundation present these recommendations to assist physicians in managing KD. This guideline can serve as a resource for basic principles of management and will evolve as new treatment strategies for this disease are identified.”

References

1. Gorelik M, Chung SA, Ardalan K, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of Kawasaki disease. Arthritis Care Res. Published online March 7, 2022. doi:10.1002/acr.24838

2. McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation. 2017;135:e927-999. doi:10.1161/CIR.0000000000000484