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Kawasaki Disease and Coronary Artery Involvement: A Narrative Review
Kruthiga Rajasekaran1, Shrimahitha Duraiyarasan2, Mayowa Adefuye3
1Research, Rajah Muthiah Medical College & Hospital, Chidambaram, IND.
Insights
Kawasaki disease, a vasculitis, can cause coronary artery aneurysms. Early diagnosis and treatment with specific therapies can prevent these dangerous cardiac complications in children.
Area of Science:
- Pediatric Cardiology
- Immunology
- Genetics
Background:
- Kawasaki disease is a systemic vasculitis affecting children, with potential for coronary artery aneurysms if untreated.
- Coronary sequelae are a significant cause of acquired heart disease in children, leading to morbidity and mortality.
- Predicting the risk of coronary artery aneurysm development remains a clinical challenge.
Purpose of the Study:
- To review the pathogenesis of coronary artery lesions in Kawasaki disease.
- To identify key genetic and immune factors involved in Kawasaki disease.
- To discuss current treatment strategies and risk stratification for preventing coronary artery aneurysms.
Main Methods:
- Clinical diagnosis of Kawasaki disease based on characteristic symptoms.
- Identification of genetic factors (ITPKC, Foxp3+, MBL2, CFH, KNG1, SERPINC1, FN1) and immune pathways.
- Review of therapeutic interventions including corticosteroids, immunoglobulins, biological agents, and immunosuppressants.
Main Results:
- Several genes, including ITPKC and MBL2, are implicated in the pathogenesis of coronary artery lesions.
- Combination therapy with aspirin, IVIG, methylprednisolone, and biological agents can prevent coronary artery aneurysms.
- Risk stratification and follow-up protocols are crucial for preventing cardiac thrombosis.
Conclusions:
- Kawasaki disease requires prompt diagnosis and management to prevent severe cardiac complications.
- Understanding the genetic and immune basis of the disease aids in developing targeted therapies.
- Adherence to established guidelines is essential for long-term patient outcomes and prevention of myocardial infarction.
Abstract:
Kawasaki disease is a systemic vasculitis with a risk of developing coronary artery lesions if left untreated. Kawasaki disease can be diagnosed clinically with classical symptoms (conjunctivitis, rash, lymphadenopathy, mucositis, edema of hands and feet), but predicting the risk of developing coronary artery aneurysm remains challenging. The coronary sequelae of Kawasaki disease have significant morbidity and mortality and are the second most common cause of acquired cardiac disease in children. Several genetic and immune factors are involved in the inflammation of coronary artery lesions in Kawasaki disease. Inositol trisphosphate 3-Kinase (ITPKC), Foxp3+, circular RNAs, mannose-binding lectin 2 (MBL2), complement factor H (CFH), kininogen 1 (KNG1), serpin family C member 1 (SERPINC1) and fibronectin 1 (FN1) are the essential genes identified in the pathogenesis of coronary artery lesions in Kawasaki disease. The addition of methylprednisolone to a combination of aspirin and intravenous immunoglobulins and biological agents like anakinra, etanercept, infliximab, and immunosuppressants like cyclosporine prevents the occurrence of coronary artery aneurysms in Kawasaki disease. Since the coronary artery lesions form the second most common cause of acquired cardiac disease in children and the incidence of myocardial infarction is a late complication, the risk stratification for coronary artery aneurysms and follow-up protocols for the prevention of cardiac thrombosis were proposed by the American Heart Association in 2017.
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