Prognosis and mortality of polymyositis and dermatomyositis patients

A Airio1, H Kautiainen, M Hakala

  • 1Rheumatism Foundation Hospital, Heinola, Finland. antti.airio@fimnet.fi

Clinical Rheumatology
|February 16, 2006
PubMed

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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