Related Experiment Video
Updated: May 16, 2026

Catheter-based Endovascular Angioplasty for Fibrosing Mediastinitis-associated Pulmonary Vein Stenosis
Published on: August 26, 2025
Management of acute and refractory Kawasaki disease
Carline E Tacke1, David Burgner, Irene M Kuipers
1Department of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Center, H7-230, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
Insights
Kawasaki disease (KD) treatment with IVIG significantly reduces coronary artery aneurysms. Steroids show promise for non-responders, but optimal management for refractory cases remains debated.
Area of Science:
- Pediatric Cardiology
- Immunology
- Rheumatology
Background:
- Kawasaki disease (KD) is a critical pediatric illness.
- High-dose intravenous immunoglobulin (IVIG) is the standard treatment for acute KD.
- IVIG significantly reduces the risk of coronary artery aneurysms.
Purpose of the Study:
- To review current evidence for acute Kawasaki disease treatment.
- To discuss management strategies for IVIG-nonresponsive KD patients.
Main Methods:
- Literature review of studies on Kawasaki disease treatment.
- Analysis of evidence for IVIG, aspirin, and adjunctive therapies.
- Discussion of emerging treatments for non-responsive cases.
Main Results:
- IVIG decreases coronary artery aneurysm incidence from 25% to <5%.
- Steroid therapy shows potential as adjunctive treatment, particularly in Asian children.
- 10-30% of patients exhibit resistance to initial IVIG therapy.
Conclusions:
- Optimal treatment for IVIG-nonresponsive KD is controversial.
- Management options include additional IVIG, corticosteroids, and other immunomodulatory agents.
- Further research is needed to establish the best approach for refractory KD.
Abstract:
Acute Kawasaki disease (KD) is treated with high-dose intravenous immunoglobulin (IVIG), which is proven to decrease the incidence of coronary artery aneurysms from 25% to less than 5%. Aspirin is also given, although the evidence base is less secure. There is increasing evidence for steroid therapy as adjunctive primary therapy with IVIG, especially in Asian children. Approximately 10-30% of patients fail to respond to the initial IVIG and are at increased risk of coronary artery aneurysms. The optimal treatment for IVIG-nonresponsive KD remains controversial. Management options include further dose(s) of IVIG, corticosteroids, TNF-α blockade, cyclosporin A, anti-IL-1 and anti-CD20 therapy. In this article, the authors review the current evidence for treatment of acute KD and discuss options for IVIG nonresponders.
Related Concept Videos
Rheumatic Heart Disease III: Medical Management
Myocarditis III: Medical Management
Acute Coronary Syndrome IV: Interprofessional Care
Mitral Stenosis III: Medical Management
Acute Kidney Injury V: Interprofessional Care
Rheumatic Heart Disease IV: Nursing Management