Related Experiment Videos
The effect of TNFalpha blockade in complicated, refractory Kawasaki disease
E V Stenbøg1, B Windelborg, A Hørlyck
1Department of Paediatrics, Aarhus University Hospital, Section SKS, DK-8200 Aarhis N, Denmark. elisabethsteinboeg@oncable.dk
Insights
Infliximab offers a promising treatment for severe Kawasaki disease (KD) unresponsive to standard therapies. This biological agent effectively reduced inflammation and coronary aneurysms in infants with refractory KD.
Area of Science:
- Pediatric Rheumatology
- Immunology
Background:
- Kawasaki disease (KD) is a childhood systemic vasculitis with elevated proinflammatory cytokines.
- Intravenous immunoglobulin (IVIG) is standard treatment, but refractory cases lack guidelines.
- Biological agents targeting cytokines are a potential future therapy.
Observation:
- Two infants with severe KD and coronary/extracoronary aneurysms showed resistance to IVIG, aspirin, and corticosteroids.
- These patients were treated with infliximab, a biologic agent targeting tumor necrosis factor alpha (TNFalpha).
Findings:
- Infliximab treatment led to prompt and sustained clinical improvement within days.
- Significant regression of coronary and extracoronary aneurysms was observed within weeks of infliximab administration.
- This suggests infliximab's efficacy in managing severe, refractory KD.
Implications:
- Infliximab may represent a viable therapeutic option for severe KD cases refractory to current treatments.
- Early intervention with biologics like infliximab could prevent long-term cardiovascular complications in KD patients.
- Further research is warranted to establish optimal protocols for biologic therapy in Kawasaki disease.
Abstract:
In Kawasaki disease (KD), a systemic vasculitis of childhood, serum levels of proinflammatory cytokines such as tumour necrosis factor alpha (TNFalpha) are elevated during the acute phase of the disease. Although the majority of children recover completely from a single dose of intravenous immunoglobulin (IVIG), the treatment is not always effective. In refractory cases of KD there are no documented treatment guidelines. A future role of biological agents directed against proinflammatory cytokines has recently been suggested by the American Heart Association (AHA). We describe two infants with severe KD, complicated by coronary as well as extracoronary aneurysms, who responded neither to repeated treatment with IVIG plus aspirin nor to corticosteroids. The children were subsequently treated with infliximab. In both cases, the effect was prompt and long-lasting. Clinical improvement was seen within a few days after the first dose, and regression of the aneurysms occurred within weeks.
Related Concept Videos
Drugs for Treatment of Crohn's Disease in IBD Using Biologic Agents: Anti-TNF
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
Heart Failure Drugs: Inhibitors of Renin-Angiotensin System
NF-κB-dependent Signaling Pathway
NF-κB-dependent Signaling Mechanism
The heterodimer of NF-κB...
Rheumatic Heart Disease I: Introduction