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. 2024 Apr 19;19(1):170.
doi: 10.1186/s13023-024-03153-6.

Elevated serum B-cell activator factor levels predict rapid progressive interstitial lung disease in anti-melanoma differentiation associated protein 5 antibody positive dermatomyositis

Yumeng Shi #  1 , Hanxiao You #  1 , Chang Liu  1 , Yulu Qiu  1 , Chengyin Lv  1 , Yujing Zhu  1 , Lingxiao Xu  1 , Fang Wang  2 , Miaojia Zhang  3 , Wenfeng Tan  4
Affiliations

Elevated serum B-cell activator factor levels predict rapid progressive interstitial lung disease in anti-melanoma differentiation associated protein 5 antibody positive dermatomyositis

Yumeng Shi et al. Orphanet J Rare Dis. .

Abstract

Background: Rapid progressive interstitial lung disease (RP-ILD) is the leading cause of anti-melanoma differentiation associated protein 5 antibody positive dermatomyositis (anti-MDA5+DM) related death. Elevated serum B-cell activating factor (BAFF) levels have been implicated in connective tissue diseases associated ILD. Here, we evaluate whether BAFF could be a prognostic biomarker for predicting RP-ILD in anti-MDA5+DM patients.

Methods: Serums were collected from 39 patients with anti-MDA5+DM (20 with RP-ILD and 19 with non-RP-ILD), 20 antisynthase syndrome (ASS) patients and 20 healthy controls (HC). BAFF concentration was measured by an enzyme-linked immunosorbent assay.

Results: Serum BAFF level was higher in anti-MDA5+DM patients than those in ASS patients and HC (3882.32 ± 1880.09 vs. 2540.89 ± 1403.04 and 2486.28 ± 767.97 pg/mL, p = 0.0056 and 0.0038, respectively). Within anti-MDA5+DM groups, RP-ILD patients exhibited higher BAFF concentration than non-RP-ILD group (4549.78 ± 1839.97 vs. 3297.28 ± 1794.69 pg/mL, p = 0.04). The BAFF concentration was positively correlated with levels of C-reactive protein (CRP), dehydrogenase (LDH) and cytokeratin (CK) in anti-MDA5+DM patients (r = 0.350, p = 0.035; r = 0.393, p = 0.016; r = 0.518, p = 0.001; respectively). The best cut-off value of BAFF concentration was 2971.5 pg/mL by ROC curve (AUC area = 0.690, p = 0.045) and BAFF > 2971.5 pg/mL was an independent risk factor for RP-ILD using multivariate analysis (OR = 9.389, 95% CI = 1.609-54.769; p = 0.013).

Conclusions: Serum BAFF could be a useful prognostic biomarker for early detecting RP-ILD risk in anti-MDA5+DM patients.

Keywords: Biomarker; Dermatomyositis; Melanoma differentiation-associated protein 5 antibody; Rapidly progressive interstitial lung disease; Serum B cell activating factor.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Serum BAFF concentration in HC, ASS and anti-MDA5+DM patients. A Serum BAFF levels in HC (n = 20), ASS (n = 20) and anti-MDA5+DM patients (n = 39); B Serum BAFF concentrations in anti-MDA5+DM patients with (n = 20) and without RP-ILD (n = 19); **p < 0.01; *p < 0.05
Fig. 2
Fig. 2
The correlation of serum BAFF and disease activity in anti-MDA5+DM. Correlation of serum BAFF levels with serum levels of CRP (A), LDH (B), CK (C), SF (D), ESR (E) in patients with anti-MDA5+DM at the first evaluation. Serum BAFF levels were determined by a specific ELISA
Fig. 3
Fig. 3
ROC curve of serum BAFF concentration in anti-MDA5+DM patients. ROC curve shows the area under the curve (AUC) of serum BAFF concentration in anti-MDA5+DM patients based on patients whether development of RP-ILD
See this image and copyright information in PMC

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