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25-011177_CG_Dermatomyositis

Dermatomyositis

Epidemiology

Julie Paik, MD, MHS 
Reviewed on February 14, 2025
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Incidence and Demographics

Dermatomyositis (DM) can occur in adults and children. Incidence of DM in the United States in adults is seen in 15.2/million person-years, with a prevalence of 21.4/100,000 people.

Adult DM affects women more than men (usually a ratio of 2:1), with incidence rates being 3.98 and 4.68 per 1,000,000 people, respectively.

The onset of DM manifests in two peak periods, diagnosed in juveniles between ages 4–14 years and in adults between ages 40–60 years. The reported mean age of disease onset is approximately 7 years and 42 years in children and adults, respectively.

Adult and juvenile DM differ in presentation, associated conditions, and prognosis. Juvenile DM has a higher incidence of vasculopathy and a higher risk of calcinosis and ulceration, though it is associated with better overall prognosis. Adults with DM are more likely to have myositis-specific antibodies and a higher risk of interstitial lung disease, amyopathic disease, and malignancy.

Higher…

Incidence and Demographics

Dermatomyositis (DM) can occur in adults and children. Incidence of DM in the United States in adults is seen in 15.2/million person-years, with a prevalence of 21.4/100,000 people.

Adult DM affects women more than men (usually a ratio of 2:1), with incidence rates being 3.98 and 4.68 per 1,000,000 people, respectively.

The onset of DM manifests in two peak periods, diagnosed in juveniles between ages 4–14 years and in adults between ages 40–60 years. The reported mean age of disease onset is approximately 7 years and 42 years in children and adults, respectively.

Adult and juvenile DM differ in presentation, associated conditions, and prognosis. Juvenile DM has a higher incidence of vasculopathy and a higher risk of calcinosis and ulceration, though it is associated with better overall prognosis. Adults with DM are more likely to have myositis-specific antibodies and a higher risk of interstitial lung disease, amyopathic disease, and malignancy.

Higher prevalence of DM has been shown in urban areas, regions with higher airborne pollution, suggesting a link with environmental factors.

About 10% of patients die of DM. The most common causes of death are malignancy and cardiopulmonary complications including inflammatory cardiomyopathy or severe Interstitial lung disease (ILD). Some factors associated with higher mortality and poor outcome are as follows: Advanced age

  • Severe muscle weakness on presentation
  • Presence of dysphagia
  • Pulmonary involvement in the form of respiratory muscle weakness or interstitial lung disease
  • Cardiac involvement
  • Underlying malignancy

References

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