Faculty of Medicine and Dentistry, Department of Medicine, University of Alberta, Edmonton, AB, T6G 2R3, Canada.
Faculty of Medicine and Dentistry, Real World Evidence Unit, University of Alberta, Edmonton, AB, T6G 2R3, Canada.
Sci Rep. 2023 Sep 30;13(1):16444. doi: 10.1038/s41598-023-43880-7.
Dermatomyositis is a rare disease characterized by progressive muscle weakness and skin rashes. Estimates of incidence and prevalence are fundamental measures in epidemiology, but few studies have been conducted on dermatomyositis. To address this knowledge gap, we conducted a population-based study to determine the contemporary incidence (between 2013 and 2019) and prevalence (2019) of adults living with dermatomyositis using administrative health data in Alberta, Canada. We also described disease-related medication use, as there are very few approved medications for the treatment of dermatomyositis, and no Canadian therapeutic guidelines. The average age- and sex-standardized annual incidence of dermatomyositis was 2.8-3.0 cases per 100,000 adults, and prevalence was 28.6 cases per 100,000 adults, which is greater than reported in other cohorts. Dermatomyositis-related medication use decreased from 73% in the first year to 46% in the eighth year after diagnosis. Glucocorticoids were the most commonly used drug class, often taken concurrently with various immunomodulatory agents; this medication use aligns with empirically-based recommendations and the few therapeutic guidelines for dermatomyositis. Considering that Alberta may have one of the highest rates of dermatomyositis among adults, further research on the burden of disease is warranted for planning within the health care system.
皮肌炎是一种罕见的疾病，其特征为进行性肌肉无力和皮疹。发病率和患病率的估计是流行病学中的基本措施，但针对皮肌炎的研究很少。为了解决这一知识空白，我们进行了一项基于人群的研究，使用加拿大艾伯塔省的行政健康数据来确定成年人中皮肌炎的当代发病率（2013 年至 2019 年）和患病率（2019 年）。我们还描述了与疾病相关的药物使用情况，因为目前只有很少的药物被批准用于治疗皮肌炎，而且加拿大也没有治疗指南。年龄和性别标准化后的皮肌炎平均年发病率为每 10 万成年人 2.8-3.0 例，患病率为每 10 万成年人 28.6 例，高于其他队列报告的发病率。皮肌炎相关药物的使用从诊断后的第一年的 73%下降到第八年的 46%。皮质类固醇是最常用的药物类别，通常与各种免疫调节剂同时使用；这种药物的使用与经验性建议和皮肌炎的少数治疗指南一致。鉴于艾伯塔省成年人中的皮肌炎发病率可能是最高的之一，因此需要进一步研究疾病负担，以便在医疗保健系统内进行规划。
