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. 2024 Aug 28;24(1):201.
doi: 10.1007/s10238-024-01462-5.

Profile of immunological biomarkers in Behcet's syndrome: a large-scale single-center real-world study

Jiachen Li #  1   2 , Feng Sun #  1   2 , Yingni Li  1   2 , Jing Zhao  1   2 , Rulin Jia  1   2 , Hongyan Wang  1   2 , Xiaohong Xiang  1   2 , Xiaolin Sun  1   2 , Chengbin Chen  3 , Haixin Xu  4 , Zhanguo Li  5   6 , Tian Liu  7   8   9
Affiliations

Profile of immunological biomarkers in Behcet's syndrome: a large-scale single-center real-world study

Jiachen Li et al. Clin Exp Med. .

Abstract

Behcet's syndrome (BS) is a vasculitis characterized by immune dysregulation. Biomarkers are valuable for assessing clinically atypical pathogenesis. We aimed to investigate the distribution of different biomarkers and their effects on the clinical features of patients with BS in a large-scale, real-world study. This is a retrospective, single-center study. In total, 502 patients diagnosed with BS were enrolled in this study. We analyzed the clinical features of this cohort and divided patients' symptoms into six categories, including mucocutaneous, articular, neurological, gastrointestinal, vascular, and ocular involvements. HLA-B51 cells, autoantibodies, and subsets of immune cells from the patients were tested. Pearson's correlation, Wilcoxon rank sum test and multivariate logistic regression were used for data analysis. Various autoantibodies were detected in the serum of 40.8% of patients with BS. The positivity rate of anti-endothelial cell antibodies (AECA) was the highest among autoantibodies and was found in 23.5% (118/502) of patients with BS. The positivity rate of HLA-B51 in patients with BS was 27.1%. Tumor necrosis factor (TNF)-α, IL-2, and IL-4 producing CD4+ T cells were positively correlated with the gastrointestinal BS. Increased IL-4+CD4+ T cell was a risk factor for gastrointestinal BS (P = 0.006, Overall rate [OR] = 2.491, 95% Confidence interval [CI]: [1.317, 5.100]). Various autoantibodies can be detected in patients with BS. HLA-B51 and AECA are the most common biomarkers. Increased IL-4+ CD4+ T cell was a risk factor for gastrointestinal involvement in BS.

Keywords: Behcet’s syndrome; Biomarkers; Clinical features; Gastrointestinal involvement.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
The positive rates of HLA-B51 and autoantibodies in patients with BS. Of all biomarkers, HLA-B51 had the highest positive rate of 27.1%. AECA was the autoantibody with highest positive rate (23.5%). The frequency of ANA is 16.7%. Positivity of other autoantibodies was less than 5%. AECA, anti-endothelial cell antibodies; ANA, antinuclear antibodies; AMA-M2, anti-mitochondrial antibodies M2
Fig. 2
Fig. 2
The positivity of HLA-B51, AECA and ANA in patients with BS with different systemic involvements. The positive rate of HLA-B51 was highest in ocular BS (34.6%) and lowest in intestinal BS (19.7%). The positive rate of AECA was highest in patients with mucocutaneous involvement alone (25.7%) and was lowest in Neuro-BS patients (6.1%). The positive rate of ANA was the highest in neuro-BS patients (21.2%) and was the lowest in vascular phenotype (11.8%)
Fig. 3
Fig. 3
The relation between different systemic involvement and the proportion of TNF-α, IFN-γ, IL-2, IL-4, IL-17 producing CD4+ T cells and Tregs among total CD4+ T cells, the absolute number of CD4+ T cells, CD8+ T cells, B cells and NK cells, and the level of serum immunoglobulins and complement in peripheral blood. The proportion of TNF-α, IL-2 and IL-4 producing CD4.+T cells were positively associated with gastrointestinal involvement of BS. The absolute number of NK cells was positively associated with ocular involvement of BS. The depth of the color represents the magnitude of the Spearman Rank correlation coefficient (Src). *P < 0.05, **P < 0.01
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