Related Experiment Video
Updated: Sep 3, 2025
qKAT: Quantitative Semi-automated Typing of Killer-cell Immunoglobulin-like Receptor Genes
Published on: March 6, 2019
Association of Genetic Polymorphisms in Kawasaki Disease with the Response to Intravenous Immunoglobulin Therapy
E Sapountzi1, L Fidani2, A Giannopoulos2
1Second Department of Pediatrics, School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, AHEPA University General Hospital, Thessaloníki, Greece. esapountzi@gmail.com.
Insights
Kawasaki disease (KD) is a childhood vasculitis. Genetic factors may predict resistance to intravenous immunoglobulin (IVIG) treatment, a key therapy for preventing coronary artery lesions.
Area of Science:
- Pediatric rheumatology
- Immunology
- Genetics
Background:
- Kawasaki disease (KD) is an acute febrile vasculitis affecting young children, with coronary artery lesions (CAL) as a primary complication.
- Standard treatment involves intravenous immunoglobulin (IVIG) and aspirin, reducing CAL incidence.
- A significant portion of patients exhibit resistance to IVIG, increasing CAL risk, with the underlying causes not fully understood.
Purpose of the Study:
- To comprehensively review genetic polymorphisms associated with IVIG resistance in Kawasaki disease.
- To discuss predictive models for IVIG unresponsiveness in KD patients.
Main Methods:
- Literature review of studies investigating genetic polymorphisms in Kawasaki disease.
- Analysis of existing models for predicting IVIG response.
Main Results:
- Several gene polymorphisms have been linked to IVIG resistance in KD.
- These genetic variations may play a role in treatment unresponsiveness.
Conclusions:
- Genetic polymorphisms are implicated in IVIG resistance in Kawasaki disease.
- Further research into these genetic factors could lead to improved prediction and personalized treatment strategies for KD.
Abstract:
Kawasaki disease (KD) is an acute febrile and systemic vasculitis disease mainly affecting children < 5 years old. Although the first case of KD was reported in 1967 and despite extensive research on KD since then, the cause of the disease remains largely unknown. The most common complications of KD are coronary artery lesions (CAL), which significantly increase the risk of coronary heart disease. The standard treatment for KD is high-dose intravenous immunoglobulin (IVIG) plus aspirin within 10 days from symptoms' appearance, which has been shown to decrease the incidence of CAL to 5-7%. Despite the benefits of IVIG, about 25% of the patients treated with IVIG develop resistance or are unresponsive to the therapy, which represents an important risk factor for CAL development. The cause of IVIG unresponsiveness has not been fully elucidated. However, the role of gene polymorphisms in IVIG response has been suggested. Herein, we comprehensively review genetic polymorphisms in KD that have been associated with IVIG resistance/unresponsiveness and further discuss available models to predict IVIG unresponsiveness.Kindly check and confirm inserted city in affiliation [1] is correctly identified.confirm.
