Different antinuclear antibody subgroups of anti-melanoma differentiation-associated gene 5-positive dermatomyositis showed variability in clinical presentation: a multicenter retrospective cohort study.
Gang Wang, Dong Yan, Leixi Xue, Chenghua Weng, Lei Zhang, Lingxiao Xu, Fenghong Yuan, Ju Li, Min Wu, Zhanyun Da, Hua Wei, Lei Zhou, Songlou Yin, Jian Wu, Yan Lu, Dinglei Su, Lin Liu, Longxin Ma, Xiaoyan Xu, Huijie Liu, Tianli Ren, Yinshan Zang, Zhichun Liu
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引用次数: 0
Abstract
Introduction: Antinuclear antibodies (ANA) are frequently positive in patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis (anti-MDA5+ DM). This study aimed to investigate the association between ANA and clinical characteristics as well as prognosis in a cohort of patients with anti-MDA5+ DM.
Material and methods: We conducted a systematic retrospective study of medical records from a Nanjing Medical University cohort of patients with myositis-associated interstitial lung disease (ILD). Various parameters were compared and analyzed between the ANA-positive group and the ANA-negative group.
Results: A total of 246 patients with anti-MDA5+ DM were enrolled in this study, with 28.5% males and 71.5% females. The median age was 53.0 years, the median disease duration was 2 months, and the median follow-up period was 12.0 months. The ANA positivity rate at baseline was 52.4% in anti-MDA5+ DM patients. The ANA-positive group showed significantly higher positivity rates of anti-Ro52 antibodies (72.9% vs. 54.7%, p = 0.003) and anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies (9.3% vs. 2.6%, p = 0.033) compared to the ANA-negative group, but lower ALT levels: 39.0 (21.5, 79.3) vs. 51.3 (36.5, 95.8), p = 0.006. No significant differences were observed between the two groups in terms of overall survival, rapidly progressive interstitial lung disease (RPILD) incidence, age, disease duration, and clinical characteristics. In a subgroup analysis of the ANA-positive group, MDA5+++ patients had a higher incidence of RPILD compared to the MDA5+ group (p = 0.028). In the ANA-negative subgroup analysis, MDA5+++ patients had a higher mortality rate and worse prognosis compared to the MDA5+ group (p = 0.026). Multivariate Cox regression analysis showed that elevated lactate dehydrogenase (LDH) levels and the presence of rapidly progressive interstitial lung disease (RPILD) were associated with poor prognosis in ANA-negative anti-MDA5+ DM patients, with hazard ratios of 1.002 (95% CI: 1.001, 1.003, p = 0.020) and 13.694 (95% CI: 15.032, 37.267, p < 0.001), respectively.
Conclusions: ANA is frequently found in patients with anti-MDA5+ DM. High titers of anti-MDA5 antibodies are associated with mortality and RPILD.
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