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. 2023 Feb;75(2):348-355.
doi: 10.1002/acr.24786. Epub 2022 Oct 6.

Incidence, Prevalence, and Mortality of Dermatomyositis: A Population-Based Cohort Study

Vanessa L Kronzer  1 , Bradly A Kimbrough  1 , Cynthia S Crowson  1 , John M Davis 3rd  1 , Marie Holmqvist  2 , Floranne C Ernste  1
Affiliations

Incidence, Prevalence, and Mortality of Dermatomyositis: A Population-Based Cohort Study

Vanessa L Kronzer et al. Arthritis Care Res (Hoboken). 2023 Feb.

Abstract

Objective: 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.

Methods: 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.

Results: 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%).

Conclusion: 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.

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Figures

Figure 1.
Figure 1.
Overall survival of Olmsted county residents with index date of dermatomyositis in 1995-2019 (solid) compared to expected rates from Minnesota lifetables (dashed)
See this image and copyright information in PMC

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