Chen Yu, Chen Yen-Fu, Chiu Li-Chung, Yu Kuang-Hui, Chan Tien-Ming
Division of Rheumatology, Allergy and Immunology, Far Eastern Memorial Hospital, New Taipei City, Taiwan.
Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, Linkou Chang Gung Memorial Hospital, Taoyuan, Taiwan.
Clin Rheumatol. 2025 Jul 15. doi: 10.1007/s10067-025-07562-1.
Patients with idiopathic inflammatory myopathy (IIM) and anti-melanoma differentiation-associated protein 5 (anti-MDA5) antibodies are at significant risk (50-90%) of developing interstitial lung disease (ILD), with a reported mortality rate of 33-60%. Notably, the coexistence of anti-MDA5 and anti-Ro52 antibodies is associated with a more severe ILD prognosis than that of anti-MDA5 antibodies alone. The aim of this study was to investigate the serological characteristics of ILD in patients with IIM who possess anti-MDA5 antibodies, with or without concurrent anti-Ro52 antibodies.
We retrospectively collected data from 32 patients diagnosed with anti-MDA5-positive IIM between May 2018 and December 2022. We reviewed these patients' clinical manifestations, serological parameters, HRCT images and outcomes. We compared the difference between the patients with and without anti-Ro-52 antibodies.
Of the 32 patients with IIM and anti-MDA5 antibodies, 16 tested positive for anti-Ro52 antibodies. Patients positive for both autoantibodies had a higher prevalence of ILD (81.25% vs. 43.75%, p = 0.028), a higher frequency of ground-glass opacity (100% vs. 42.85%, p = 0.002) of HRCT findings, higher C-reactive protein levels (2.9 vs. 1.4 mg/L, p = 0.038), and were older (54.2 vs. 42.1 years, p = 0.021) than those without. Patients who were positive for the coexistence of anti-MDA5 and anti-Ro52 antibodies tended to have a lower survival rate, although this difference was not statistically significant (p = 0.124).
Individuals with IIM coexistence of anti-MDA5 and anti-Ro52 antibodies may experience a greater inflammatory response, higher ILD frequency, and poorer prognosis than those without.
• The coexistence of anti-MDA5 and anti-Ro52 antibodies in a small cohort of patients with IIM was found to be associated with a higher overall inflammatory features, including the presence of interstitial lung disease. • Coexistence of these antibodies and interstitial lung disease particularly relating to the radiological findings of ground glass opacities can be helpful in providing insights into evaluation and diagnostic pathways for clinicians. • A trend for a lower survival rate was observed for patients with the presence of these two antibodies than anti-MDA5 antibody alone.
特发性炎性肌病(IIM)患者及抗黑色素瘤分化相关蛋白5(抗MDA5)抗体阳性患者发生间质性肺疾病(ILD)的风险很高(50%-90%),报告的死亡率为33%-60%。值得注意的是,抗MDA5和抗Ro52抗体共存与单独抗MDA5抗体相比,ILD预后更严重。本研究的目的是调查具有抗MDA5抗体的IIM患者中ILD的血清学特征,无论是否同时存在抗Ro52抗体。
我们回顾性收集了2018年5月至2022年12月期间诊断为抗MDA5阳性IIM的32例患者的数据。我们回顾了这些患者的临床表现、血清学参数、HRCT图像和结局。我们比较了抗Ro-52抗体阳性和阴性患者之间的差异。
在32例IIM及抗MDA5抗体阳性患者中,16例抗Ro52抗体检测呈阳性。两种自身抗体均阳性的患者ILD患病率更高(81.25%对43.75%,p = 0.028),HRCT表现中磨玻璃影的出现频率更高(100%对42.85%,p = 0.002),C反应蛋白水平更高(2.9对1.4mg/L,p = 0.038),且年龄更大(54.2对42.1岁,p = 0.021)。抗MDA5和抗Ro52抗体共存阳性的患者生存率往往较低,尽管这种差异无统计学意义(p = 0.124)。
与不共存抗MDA5和抗Ro52抗体的IIM患者相比,共存这两种抗体的患者可能经历更大的炎症反应、更高的ILD发生率和更差的预后。
• 在一小群IIM患者中,发现抗MDA5和抗Ro52抗体共存与更高的整体炎症特征相关,包括间质性肺疾病的存在。• 这些抗体的共存及与磨玻璃影放射学表现相关的间质性肺疾病,有助于为临床医生提供评估和诊断途径的见解。• 观察到同时存在这两种抗体的患者比单独抗MDA5抗体阳性患者的生存率有降低趋势。