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Immunophenotype of Kawasaki Disease: Insights into Pathogenesis and Treatment Response
Aikaterini Agrafiotou1, Evdoxia Sapountzi2, Angeliki Margoni3
1Division of Pediatric Rheumatology, Department of Pediatrics, ATTIKON General Hospital, National and Kapodistrian University of Athens, 12462 Athens, Greece.
Insights
Kawasaki disease (KD) immunophenotyping reveals key immune cell and protein biomarkers. These insights aid early diagnosis, predict treatment response, and advance understanding of this inflammatory condition.
Area of Science:
- Immunology
- Pediatric Rheumatology
- Vascular Biology
Background:
- Kawasaki disease (KD) is a critical pediatric inflammatory condition causing vasculitis and potential coronary artery lesions.
- Diagnosis relies on clinical symptoms, often delayed by overlap with other febrile illnesses, impacting treatment timeliness.
- A significant portion of patients exhibit resistance to standard therapies, necessitating alternative treatment strategies.
Purpose of the Study:
- To systematically review the last decade of research on Kawasaki disease immunophenotypes.
- To identify biomarkers associated with KD pathogenesis and treatment response.
- To consolidate findings on immune system involvement in KD.
Main Methods:
- Comprehensive literature review of studies published in the last 10 years.
- Focus on immunophenotyping techniques, primarily flow cytometry.
- Analysis of biomarkers related to innate and adaptive immunity, cytokines, chemokines, and complement receptors.
Main Results:
- Immunophenotyping has identified critical roles for both innate and adaptive immune cells in KD pathogenesis.
- Specific cell surface proteins and secreted factors, including cytokines and chemokines, are implicated in disease development.
- Biomarkers are emerging as potential indicators for predicting treatment efficacy in KD patients.
Conclusions:
- Understanding KD immunophenotypes is crucial for advancing diagnostic accuracy and therapeutic strategies.
- Biomarkers identified through immunophenotyping can guide early intervention and personalized treatment approaches.
- Further research into these biomarkers may unlock novel therapeutic targets for resistant KD cases.
Abstract:
Kawasaki disease (KD) is a systematic inflammatory condition that results in vasculitis and possible progression to the development of coronary artery lesions if left untreated. Disease pathogenesis is not fully understood, and diagnosis is based on clinical symptoms, with limited reliability considering that KD progression is time sensitive. This is further complicated by the shared clinical characteristics with other febrile diseases. Early diagnosis and prompt treatment start are associated with good prognosis in most patients. However, up to 20% of patients are resistant to available therapeutic agents and would benefit from alternative regimens. Therefore, identification of biomarkers that can provide insights on disease pathogenesis are necessary to enable early diagnosis and initiation of treatment, as well as to predict treatment responses. To this end, immunophenotyping, most commonly by flow cytometry, has been crucial in identifying central factors in KD pathogenesis. The available literature on such factors is vast and may include contradictory findings. Therefore, we aimed to summarize the available literature of the last decade on the immunophenotype of KD, focusing on biomarkers associated with disease pathogenesis and those associated with treatment response. Our review highlights the role of cells of both the innate and adaptive immune system in disease pathogenesis, as well as the role of various secreted and cell surface proteins, including inflammatory cytokines, chemokines, complement receptors, and chemoattractants both in KD pathogenesis and in treatment response.
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