Response of refractory Kawasaki disease to pulse steroid and cyclosporin A therapy
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Published on: November 8, 2015
1Division of Cardiology, Immunology and Rheumatology, St. Louis Children's Hospital, MO, USA.
Insights
This study details a child with severe Kawasaki disease (KD) that resisted initial treatment. Aggressive immunosuppression with corticosteroids and cyclosporin A proved effective for refractory KD.
Area of Science:
- Pediatrics
- Cardiology
- Immunology
Background:
- Kawasaki disease (KD) is an acute febrile vasculitis affecting young children.
- Coronary artery aneurysms are a major complication of KD.
- Intravenous immunoglobulin (IVIG) is the standard first-line therapy.
Observation:
- A pediatric patient presented with aggressive and protracted KD.
- The patient developed coronary aneurysms, myocarditis, pericarditis, and valvular insufficiency.
- Initial IVIG treatment yielded only a transient response.
Findings:
- Pulse corticosteroids provided temporary improvement.
- A combination of high-dose corticosteroids and cyclosporin A led to disease remission.
- This case highlights a potential treatment pathway for refractory KD.
Implications:
- Aggressive immunosuppressive therapy may be crucial for managing severe, refractory Kawasaki disease.
- High-dose corticosteroids combined with cyclosporin A show promise in treatment-resistant cases.
- Further research into advanced immunosuppressive strategies for KD is warranted.
Abstract:
We describe a child with aggressive and protracted Kawasaki disease with coronary aneurysms, myocarditis, pericarditis and valvular insufficiency, despite repeated administration of intravenous immunoglobulin. After a transient response to pulse corticosteroids, his disease ultimately subsided with combination therapy with pulse and high dosage corticosteroids and cyclosporin A. Aggressive immunosuppressive therapy with high dosage corticosteroids and cyclosporin A may be beneficial in patients with refractory Kawasaki disease.
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