Lv Chengyin, You Hanxiao, Xu Lingxiao, Wang Lei, Yuan Fenghong, Li Ju, Wu Min, Zhou Shiliang, Da Zhanyun, Qian Jie, Wei Hua, Yan Wei, Zhou Lei, Wang Yan, Yin Songlou, Zhou Dongmei, Wu Jian, Lu Yan, Su Dinglei, Liu Zhichun, Liu Lin, Ma Longxin, Xu Xiaoyan, Zang Yinshan, Liu Huijie, Ren Tianli, Wang Fang, Zhang Miaojia, Tan Wenfeng
C. Lv, MD, H. You, MD, PhD, L. Xu, MD, PhD, L. Wang, MD, PhD, M. Zhang, MD, W. Tan, MD, PhD, Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing.
F. Yuan, MD, Department of Rheumatology and Immunology, The Affiliated Wuxi People's Hospital of Nanjing Medical University, Wuxi.
J Rheumatol. 2023 Feb;50(2):219-226. doi: 10.3899/jrheum.220139. Epub 2022 Jun 15.
Interstitial lung disease (ILD) is a common extramuscular complication contributing to significant morbidity and mortality in patients with dermatomyositis (DM) who are positive for antimelanoma differentiation-associated gene 5 antibody (anti-MDA5+). We conducted this study to investigate the association of anti-Ro52 antibodies with clinical characteristics and prognosis in patients with anti-MDA5+ DM.
We assessed a cohort of 246 patients with anti-MDA5+ DM. To calculate hazard ratios and 95% CIs for rapidly progressive ILD (RP-ILD) and death while controlling for potential confounders, variables selected by univariate Cox regression analysis were included in a multivariate Cox regression model with the stepwise forward-selection method. A 2-tailed analysis with < 0.05 was considered to be statistically significant.
A total of 246 patients with anti-MDA5+ DM were enrolled; 70 patients were male, and the patient group had an average age of 53.1 (12.4) years. Anti-Ro52 was present in 64.2% (158/246) patients. Patients with anti-MDA5+ DM who were positive for anti-Ro52 had a higher rate of RP-ILD (log-rank < 0.001) and a higher mortality rate (log-rank = 0.01). For patients with anti-MDA5+ DM who were positive for anti-Ro52, those with a short disease course and high inflammation were at increased risk of RP-ILD and death. The appearance of active rash was an independent protective factor of death.
Anti-Ro52 antibodies were highly prevalent in patients with anti-MDA5+ DM, and their coexistence correlated with a higher rate of RP-ILD and mortality. Patients with a short disease course, with increased inflammation, and without rash were more likely to have a poor prognosis.
间质性肺病（ILD）是抗黑色素瘤分化相关基因5抗体阳性（抗MDA5+）的皮肌炎（DM）患者常见的肌肉外并发症，可导致显著的发病率和死亡率。我们开展这项研究以调查抗Ro52抗体与抗MDA5+ DM患者临床特征及预后的相关性。
我们评估了一组246例抗MDA5+ DM患者。为在控制潜在混杂因素的同时计算快速进展性ILD（RP-ILD）和死亡的风险比及95%置信区间，将单变量Cox回归分析选择的变量纳入多变量Cox回归模型，采用逐步向前选择法。双侧分析，P<0.05被认为具有统计学意义。
共纳入246例抗MDA5+ DM患者；70例为男性，患者组平均年龄为53.1（12.4）岁。64.2%（158/246）的患者抗Ro52阳性。抗Ro52阳性的抗MDA5+ DM患者发生RP-ILD的比率更高（对数秩检验P<0.001），死亡率更高（对数秩检验P=0.01）。对于抗Ro52阳性的抗MDA5+ DM患者，病程短且炎症程度高的患者发生RP-ILD和死亡的风险增加。活动性皮疹的出现是死亡的独立保护因素。
抗Ro52抗体在抗MDA5+ DM患者中高度流行，其共存与更高的RP-ILD发生率和死亡率相关。病程短、炎症增加且无皮疹的患者预后更差。
