Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden; Department of Gastro, Dermatology and Rheumatology, Karolinska University Hospital, Stockholm, Sweden.
Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden; Department of Gastro, Dermatology and Rheumatology, Karolinska University Hospital, Stockholm, Sweden.
Semin Arthritis Rheum. 2024 Oct;68:152529. doi: 10.1016/j.semarthrit.2024.152529. Epub 2024 Aug 8.
To study the trajectories of changes in damage over time and explore associations with autoantibody defined subgroups using a large international cohort of patients with idiopathic inflammatory myopathies (IIM).
Data from the MYONET registry, including patients who were tested for autoantibodies and had at least one assessment of damage using the Myositis Damage Index (MDI), were analyzed. Patients were sub-grouped according to their autoantibody profiles (myositis-specific, myositis-associated, or seronegative). The index date was defined as the time point for the first registered MDI assessment. The longitudinal trajectories of damage with autoantibody status as the main predictor were analyzed using linear mixed models.
A total of 757 adult patients were included in this study. Each year of disease duration since diagnosis had an estimated MDI score increase of 0.16 units for the seronegative group (reference). Compared with the seronegative group as reference, patients with dermatomyositis-specific autoantibodies developed less damage per year of follow-up since diagnosis (average 0.08 less score, P = 0.04), whereas patients with anti-PM/Scl autoantibodies developed more damage per year of follow-up since diagnosis (average 0.28 higher score, P = 0.03) independent of sex and age at diagnosis. The seronegative subgroup and the immune-mediated necrotizing myopathy autoantibody subgroup had the strongest correlation between severity of muscle damage and HAQ-DI scores at five years of follow-up, rho=0.84, P < 0.001 and rho=0.72, P < 0.001, respectively.
Our study is the first to describe patterns and trajectories of change in damage over time in relation to autoantibody defined subgroups in a large international multicenter cohort of patients with IIM. Patients with anti-PM/Scl scored a greater extent of damage, whereas patients with dermatomyositis-specific antibodies had less damage than seronegative patients. Severity in muscle damage had moderate to strong correlation with functional disability among the IMNM and seronegative subgroups with lower correlations for the other subgroups. These findings suggest that autoantibodies may be useful predictors of long-term damage.
通过对特发性炎性肌病（IIM）患者的大型国际队列进行研究，分析随着时间推移损伤变化的轨迹，并探讨与自身抗体定义的亚组之间的关联。
对 MYONET 注册中心的数据进行分析，这些数据包括接受自身抗体检测且至少有一次使用肌炎损伤指数（MDI）评估损伤的患者。根据自身抗体谱（肌炎特异性、肌炎相关性或血清阴性）将患者分为亚组。指数日期定义为首次登记 MDI 评估的时间点。使用线性混合模型分析以自身抗体状态为主要预测因子的损伤的纵向轨迹。
本研究共纳入 757 例成年患者。血清阴性组（参考组）从诊断到疾病的每一年，MDI 评分的估计增加 0.16 分。与血清阴性组作为参考相比，皮肌炎特异性自身抗体患者的每年损伤进展程度较低（平均少 0.08 分，P = 0.04），而抗 PM/Scl 自身抗体患者的每年损伤进展程度较高（平均高 0.28 分，P = 0.03），且与性别和诊断时的年龄无关。在五年随访中，血清阴性亚组和免疫介导性坏死性肌病自身抗体亚组与 HAQ-DI 评分之间的肌肉损伤严重程度具有最强的相关性，rho=0.84，P<0.001 和 rho=0.72，P<0.001。
本研究首次描述了在大型国际多中心特发性炎性肌病患者队列中，与自身抗体定义的亚组相关的时间推移中损伤变化的模式和轨迹。抗 PM/Scl 评分的患者损伤程度更大，而皮肌炎特异性抗体患者的损伤程度比血清阴性患者的损伤程度小。在免疫介导性坏死性肌病和血清阴性亚组中，肌肉损伤的严重程度与功能残疾之间存在中度至高度相关性，而在其他亚组中相关性较低。这些发现表明，自身抗体可能是长期损伤的有用预测因子。
