Paediatric Rheumatology
Clinical and diagnostic characteristics of arterial involvement in paediatric Behçet’s disease
M. Wen1, M. Li2, L. Wang3, Y. Xu4, D. Zhang5, J. Zhu6, F. Wu7, J. Lai8
- Department of Rheumatology and Immunology, Capital Center for Children’s Health, Capital Medical University, Beijing, China.
- Department of Rheumatology and Immunology, Capital Center for Children’s Health, Capital Medical University, Beijing, China.
- Department of Paediatrics, General Hospital of Ningxia Medical University, Yinchuan, China.
- Department of Rheumatology and Immunology, Capital Center for Children’s Health, Capital Medical University, Beijing, China.
- Department of Rheumatology and Immunology, Capital Center for Children’s Health, Capital Medical University, Beijing, China.
- Department of Rheumatology and Immunology, Capital Center for Children’s Health, Capital Medical University, Beijing, China.
- Department of Rheumatology and Immunology, Capital Center for Children’s Health, Capital Medical University, Beijing, China.
- Department of Rheumatology and Immunology, Capital Center for Children’s Health, Capital Medical University, Beijing, China. laijm99@sina.com
CER19489
Paediatric Rheumatology
PMID: 42446644 [PubMed]
Received: 04/11/2025
Accepted : 27/03/2026
In Press: 09/07/2026
Abstract
OBJECTIVES:
To explore the clinical features, diagnosis, treatment and prognosis of arterial involvement in paediatric Behçet’s disease (BD) for clinical reference.
METHODS:
A retrospective cohort study analysed 76 paediatric BD patients (January 2013 - May 2024). Nineteen with arterial involvement were the experimental group, and 57 without vascular involvement were the control group.
RESULTS:
The experimental group mainly involved medium-sized (15/19, 78.95%), large-sized (13/19, 68.42%) or both (9/19, 47.37%) arteries, most commonly abdominal aorta (8/19, 42.11%), pulmonary artery (7/19, 36.84%) and femoral artery (7/19, 36.84%). Notably, we found a high prevalence of coronary artery involvement, exclusively manifesting as left main coronary artery dilation. Lesions were mainly wall thickening (9/19, 47.37%), lumen dilation (8/19, 42.11%) and stenosis (7/19, 36.84%). Compared with the control group, it had later onset age (11.0 vs. 7.0 years), shorter disease duration (6.0 vs. 24.0 months), and higher incidences of fever, multi-organ involvement (neurological, renal, cardiac), and elevated inflammatory markers including C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). It also had more intensive treatment and surgery but had significantly higher rates of cerebrovascular accidents (15.79% vs. 0%), cardiac complications (31.58% vs. 0%), and mortality (10.53% vs. 0%).
CONCLUSIONS:
Paediatric BD with arterial involvement is a severe phenotype with a poor prognosis, characterized by intense inflammation and multi-organ damage. This underscores the critical need for early identification, aggressive treatment, and close monitoring to improve long-term outcomes.
