B-cell receptor repertoire remodeling in Kawasaki disease is associated with clonal expansion and distinct repertoire features in coronary artery lesions
- 1Department of Immunology, Center of Immunomolecular Engineering, Zunyi Medical University
- 2Department of Cardiology, the Affiliated Children’s Hospital of Kunming Medical University
Abstract
Background: Kawasaki disease (KD) is an acute systemic vasculitis in children, but the characteristics of the B-cell receptor (BCR) repertoire during acute disease and their association with coronary artery lesions (CALs) remain poorly understood.
Methods: High-throughput sequencing of BCR heavy-chain (BCR-H) CDR3 regions was performed in 22 children, including 9 healthy controls and 13 patients with KD, of whom 9 had no CAL and 4 had CAL. Repertoire diversity, clonal expansion, immunoglobulin isotype distribution, V–J gene usage, CDR3 characteristics, and inter-individual and cross-isotype repertoire sharing were systematically evaluated.
Results: KD exhibited reduced BCR repertoire diversity, with a significant decrease in IgM diversity. The repertoire showed increased contributions of high-frequency and hyperexpanded clonotypes, with the most pronounced expansion in patients with CAL. IgM displayed the strongest clonal remodeling, characterized by reduced low-frequency and increased highly abundant clonotypes. KD also showed selective alterations in IGHV usage and V–J gene pairing. CAL was associated with increased IGHA usage and changes in CDR3 amino acid composition. Substantial inter-individual CDR3 sharing was observed among KD patients, and 20 recurrent clonotypes absent from healthy controls were identified as candidate KD-associated sequences. Cross-isotype CDR3 sharing also tended to be higher in KD, particularly among patients with CAL.
Conclusions: Acute KD is characterized by substantial BCR repertoire remodeling, marked by selective IgM clonal expansion, repertoire convergence, and CAL-associated IgA-biased remodeling. These findings provide new insights into humoral immune dysregulation in KD and identify candidate B-cell clonotypes for further investigation of disease pathogenesis and coronary involvement.
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BibTeX
@article{xu2026bcell,
title = {{B-cell receptor repertoire remodeling in Kawasaki disease is associated with clonal expansion and distinct repertoire features in coronary artery lesions}},
author = {Yuanyuan Xu and Xixiong Lin and Yanfei Chen and Xing Zhang and Long Ma and Xinsheng Yao and Jun Li},
journal = {Frontiers in Immunology},
year = {2026},
month = oct,
doi = {10.3389/fimmu.2026.1994376},
url = {https://www.frontiersin.org/articles/10.3389/fimmu.2026.1994376/full},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - B-cell receptor repertoire remodeling in Kawasaki disease is associated with clonal expansion and distinct repertoire features in coronary artery lesions
AU - Yuanyuan Xu
AU - Xixiong Lin
AU - Yanfei Chen
AU - Xing Zhang
AU - Long Ma
AU - Xinsheng Yao
AU - Jun Li
PY - 2026
DA - 2026/10/14
JO - Frontiers in Immunology
DO - 10.3389/fimmu.2026.1994376
UR - https://www.frontiersin.org/articles/10.3389/fimmu.2026.1994376/full
AB - Background: Kawasaki disease (KD) is an acute systemic vasculitis in children, but the characteristics of the B-cell receptor (BCR) repertoire during acute disease and their association with coronary artery lesions (CALs) remain poorly understood. Methods: High-throughput sequencing of BCR heavy-chain (BCR-H) CDR3 regions was performed in 22 children, including 9 healthy controls and 13 patients with KD, of whom 9 had no CAL and 4 had CAL. Repertoire diversity, clonal expansion, immunoglobulin isotype distribution, V–J gene usage, CDR3 characteristics, and inter-individual and cross-isotype repertoire sharing were systematically evaluated. Results: KD exhibited reduced BCR repertoire diversity, with a significant decrease in IgM diversity. The repertoire showed increased contributions of high-frequency and hyperexpanded clonotypes, with the most pronounced expansion in patients with CAL. IgM displayed the strongest clonal remodeling, characterized by reduced low-frequency and increased highly abundant clonotypes. KD also showed selective alterations in IGHV usage and V–J gene pairing. CAL was associated with increased IGHA usage and changes in CDR3 amino acid composition. Substantial inter-individual CDR3 sharing was observed among KD patients, and 20 recurrent clonotypes absent from healthy controls were identified as candidate KD-associated sequences. Cross-isotype CDR3 sharing also tended to be higher in KD, particularly among patients with CAL. Conclusions: Acute KD is characterized by substantial BCR repertoire remodeling, marked by selective IgM clonal expansion, repertoire convergence, and CAL-associated IgA-biased remodeling. These findings provide new insights into humoral immune dysregulation in KD and identify candidate B-cell clonotypes for further investigation of disease pathogenesis and coronary involvement.
SN - 1664-3224
ER -