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Prognosis and mortality of polymyositis and dermatomyositis patients
A Airio1, H Kautiainen, M Hakala
1Rheumatism Foundation Hospital, Heinola, Finland. antti.airio@fimnet.fi
Abstract:
The objective of this study was to assess the long-term outcome of polymyositis (PM) and dermatomyositis (DM) and the factors predictive of this outcome in a nationwide series in Finland. One hundred and seventy-six patients with PM and 72 patients with DM diagnosed in Finland in 1969-1985 were selected from the national hospital discharge register according to the diagnostic criteria of Bohan and Peter and followed up until death or till the end of August 1995. Gender, age, delay of therapy, serum creatine kinase, erythrocyte sedimentation rate, initial dose of corticosteroids and duration of cytostatic therapy were assessed as factors prognostic of death. The 5-year survival rate for PM was 75% [95% confidence interval (CI): 68-81%] and that for DM 63% (50-73%), and the respective 10-year survival rates were 55% (47-62%) and 53% (41-64%). The median survival for polymyositis was 11.0 years (95% CI: 9.5-13.3) and that for DM 12.3 years (5.5-20.7). The standardized mortality ratio for the combined group of PM and DM showed approximately threefold mortality compared to the general population. Compared to PM patients, the subjects with DM had a 1.47-fold (95% CI: 0.99-2.12) age- and sex-adjusted risk of mortality. Except for age in both groups and the delay in diagnosis in the PM group, no other individual factor reached significance as a predictor of death. However, cancer had a hazard ratio (HR) of 2.16 for death (95% CI: 0.95-4.50) in the DM group and 1.99 (95% CI: 1.01-3.94) in the PM group. A comparison of the causes of death in the PM and DM groups showed that the patients with DM had a greater risk of dying from cancer (age-adjusted HR 5.11, 95% CI: 2.31-11.3). According to this nationwide analysis of survival and its prognostic factors in patients with PM and DM, the latter group had an increased age-adjusted risk for mortality compared to the former. The difference seems to be mostly explained by the fact that the patients with DM had a higher risk of dying from cancer.
Insights
This study found that patients with dermatomyositis (DM) have a higher mortality risk than those with polymyositis (PM), largely due to an increased risk of dying from cancer. Long-term survival rates for both conditions remain significantly lower than the general population.
Area of Science:
- Rheumatology and Immunology
- Epidemiology and Public Health
- Oncology
Background:
- Polymyositis (PM) and dermatomyositis (DM) are rare idiopathic inflammatory myopathies with significant long-term morbidity and mortality.
- Understanding survival rates and prognostic factors is crucial for patient management and healthcare resource allocation.
- Previous studies have varied in their findings regarding long-term outcomes and specific risk factors for PM and DM.
Purpose of the Study:
- To evaluate the long-term survival outcomes of polymyositis (PM) and dermatomyositis (DM) patients in Finland.
- To identify factors that predict mortality in a nationwide cohort of PM and DM patients.
- To compare mortality risks and causes of death between PM and DM.
Main Methods:
- A nationwide cohort of 176 PM and 72 DM patients diagnosed in Finland between 1969-1985 was identified.
- Patients were followed up until death or August 1995, using national hospital discharge registers and diagnostic criteria by Bohan and Peter.
- Prognostic factors assessed included gender, age, treatment delay, serum creatine kinase, ESR, corticosteroid dose, and cytostatic therapy duration.
Main Results:
- The 5-year survival rates were 75% for PM and 63% for DM; 10-year rates were 55% for PM and 53% for DM.
- The standardized mortality ratio for the combined PM and DM group was approximately threefold that of the general population.
- Dermatomyositis (DM) patients had a 1.47-fold increased age- and sex-adjusted mortality risk compared to polymyositis (PM) patients, primarily driven by a 5.11-fold higher risk of cancer-related death.
Conclusions:
- Patients with dermatomyositis (DM) face a significantly higher age-adjusted mortality risk than those with polymyositis (PM).
- Cancer is a major cause of death in DM patients, contributing significantly to the increased mortality observed in this group.
- Age and delayed diagnosis were significant predictors of death in PM, while cancer risk was elevated in both groups, particularly DM.
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