Kridin Khalaf, Kridin Mouhammad, Amital Howard, Watad Abdulla, Khamaisi Mogher
Department of Dermatology, Rambam Health Care Campus, Haifa, Israel.
Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
Isr Med Assoc J. 2020 Oct;22(10):623-627.
The reported mortality rates of patients with polymyositis and dermatomyositis are highly variable worldwide. The excess mortality of patients with polymyositis/dermatomyositis has not been evaluated in an Israeli population.
To investigate the overall mortality in a large and well-established cohort of patients with polymyositis/dermatomyositis as compared to the mortality expected in the matched general population in a tertiary medical center.
In this retrospective cohort study, the mortality of 166 patients with polymyositis/dermatomyositis was compared to age- and sex-matched control subjects in the general population. All-cause standardized mortality ratios (SMRs) were estimated.
Overall, 47 (28.3%) deaths were observed among patients with polymyositis/dermatomyositis during a mean follow-up period of 5.8 ± 4.8 years, which was 7 times higher than in the control group (SMR 7.4, 95% confidence interval [95%CI] 5.5-9.8). The SMRs were comparable in patents with polymyositis (7.7, 95%CI 4.8-12.3) and dermatomyositis (7.2, 95%CI 5.0-10.3). The 1-, 5-, 10-, and 15-year overall survival rates were 90.0%, 82.8%, 51.5%, and 26.1%, respectively, in patients with polymyositis, and 80.3%, 59.6%, 40.0%, and 17.1%, respectively, in patients with dermatomyositis.
The overall mortality among Israeli patients with polymyositis/dermatomyositis is 7.4 times greater than for the general population. Although long-term mortality was comparable between patients with dermatomyositis and polymyositis, patients in the former group died at a notably earlier stage.
据报道，全球多发性肌炎和皮肌炎患者的死亡率差异很大。以色列人群中多发性肌炎/皮肌炎患者的额外死亡率尚未得到评估。
在一家三级医疗中心，调查一个大型且成熟的多发性肌炎/皮肌炎患者队列的总体死亡率，并与匹配的普通人群的预期死亡率进行比较。
在这项回顾性队列研究中，将166例多发性肌炎/皮肌炎患者的死亡率与普通人群中年龄和性别匹配的对照者进行比较。估计全因标准化死亡率（SMR）。
总体而言，在平均随访期5.8±4.8年期间，多发性肌炎/皮肌炎患者中有47例（28.3%）死亡，这比对照组高7倍（SMR 7.4，95%置信区间[95%CI] 5.5 - 9.8）。多发性肌炎患者（7.7，95%CI 4.8 - 12.3）和皮肌炎患者（7.2，95%CI 5.0 - 10.3）的SMR相当。多发性肌炎患者的1年、5年、10年和15年总生存率分别为90.0%、82.8%、51.5%和26.1%，皮肌炎患者分别为80.3%、59.6%、40.0%和17.1%。
以色列多发性肌炎/皮肌炎患者的总体死亡率比普通人群高7.4倍。尽管皮肌炎患者和多发性肌炎患者的长期死亡率相当，但前一组患者死亡时间明显更早。
