Mayo Clinic, Rochester, Minnesota.
Karolinska Institutet, Stockholm, Sweden.
Arthritis Care Res (Hoboken). 2023 Feb;75(2):348-355. doi: 10.1002/acr.24786. Epub 2022 Oct 6.
We aimed to determine the population-based incidence, prevalence, and mortality of dermatomyositis (DM) using European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) criteria.
This population-based cohort study included incident DM from January 1, 1995 to December 31, 2019. We manually reviewed all individuals with at least 1 code for DM or polymyositis to determine if they met EULAR/ACR criteria, subspecialty physician diagnosis, and/or Bohan and Peter criteria. We age- and sex-adjusted incidence and prevalence estimates to the US non-Hispanic White year 2000 population and estimated prevalence on January 1, 2015. Standardized mortality ratios (SMRs) with 95% confidence intervals (95% CIs) compared observed to expected mortality adjusting for age, sex, and year.
We identified 40 cases of verified DM, with 29 cases incident in Olmsted County from 1995 to 2019. The mean age was 57 years, 26 (90%) were female, and 12 (41%) had clinically amyopathic DM (CADM). The median follow-up time was 8.2 years. The overall adjusted incidence of DM was 1.1 (95% CI 0.7-1.5) per 100,000 person-years, and prevalence was 13 (95% CI 6-19) per 100,000. The SMR was significantly elevated among the myopathic DM cases (3.1 [95% CI 1.1-6.8]) but not CADM cases (1.1 [95% CI 0.2-3.3]). The positive predictive value of ≥2 DM codes was only 40 of 82 (49%).
This population-based study found that DM incidence and prevalence were higher than previously reported. Mortality was significantly elevated for myopathic DM but not for CADM.
本研究旨在采用欧洲抗风湿病联盟（EULAR）/美国风湿病学会（ACR）标准，确定基于人群的皮肌炎（DM）发病率、患病率和死亡率。
本基于人群的队列研究纳入了 1995 年 1 月 1 日至 2019 年 12 月 31 日期间的新发 DM 患者。我们手动查阅了所有至少有 1 个 DM 或多发性肌炎编码的患者的记录，以确定其是否符合 EULAR/ACR 标准、专科医生的诊断标准，以及 Bohan 和 Peter 标准。我们将发病率和患病率估计值调整为美国非西班牙裔白人 2000 年人口，并估计了 2015 年 1 月 1 日的患病率。采用标准化死亡率比（SMR）及其 95%置信区间（95%CI），比较了年龄、性别和年份校正后的观察死亡率与预期死亡率。
我们共发现 40 例确诊 DM 患者，其中 29 例为奥姆斯特德县（Olmsted County） 1995 年至 2019 年的新发病例。患者的平均年龄为 57 岁，26 例（90%）为女性，12 例（41%）为临床无肌病性皮肌炎（CADM）。中位随访时间为 8.2 年。DM 的总调整发病率为 1.1（95%CI 0.7-1.5）/100,000 人年，患病率为 13（95%CI 6-19）/100,000。肌病性 DM 病例的 SMR 显著升高（3.1[95%CI 1.1-6.8]），而 CADM 病例的 SMR 无显著升高（1.1[95%CI 0.2-3.3]）。≥2 个 DM 编码的阳性预测值仅为 82 例中的 40 例（49%）。
本基于人群的研究发现，DM 的发病率和患病率高于之前的报道。肌病性 DM 的死亡率显著升高，但 CADM 病例的死亡率无显著升高。
