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Morbidity and mortality in adult polymyositis and dermatomyositis
1Département de Médecine Interne, CHU de Rouen, France. isabelle.marie@chu-rouen.fr
Abstract:
Before the use of corticosteroids, the prognosis for polymyositis/dermatomyositis (PM/DM) was extremely poor. To date, although overall prognosis appears to be better, PM and DM are still considered to be associated with increased morbidity, primarily related to severe muscle weakness and visceral involvement. Recent series underline that only 20% to 40% of treated patients will achieve PM/DM remission, whereas 60% to 80% will experience a polycyclic or chronic, continuous course of the disease. PM/DM further continues to have a great impact on life in medium- and long-term follow-up, as up to 80% of treated patients are still disabled (using Health Assessment Questionnaire scores). The overall mortality ratio in PM/DM patients also remains threefold higher compared with the general population, with cancer, lung, and cardiac complications and infections being the most common causes of deaths. Predictive factors for a poor prognosis in PM/DM patients are older age, involvement of lung and cardiac systems, dysphagia, cancer, and serum myositis-specific antibodies (including coexistent presence of anti-Ro52 and anti-Jo1 antibodies, anti-signal recognition particle antibody, anti-155/140, and anti-CADM-140 antibodies).
Insights
Polymyositis/dermatomyositis (PM/DM) still causes significant disability and increased mortality despite treatment. Many patients experience chronic disease courses, highlighting the need for improved management strategies for these inflammatory myopathies.
Area of Science:
- Rheumatology
- Immunology
- Neurology
Background:
- Polymyositis/dermatomyositis (PM/DM) historically had a poor prognosis before corticosteroid use.
- Current treatments improve outcomes, but PM/DM remains associated with significant morbidity and mortality.
Purpose of the Study:
- To review the current prognosis, morbidity, and mortality associated with polymyositis/dermatomyositis.
- To identify predictive factors for a poor prognosis in PM/DM patients.
Main Methods:
- Literature review of recent series and clinical data on PM/DM patients.
- Analysis of patient outcomes including remission rates, long-term disability, and mortality.
- Identification of clinical and serological factors associated with disease severity.
Main Results:
- Only 20-40% of treated patients achieve remission; 60-80% have chronic or relapsing disease.
- Up to 80% of patients experience long-term disability, and mortality is threefold higher than the general population.
- Cancer, lung/cardiac complications, and infections are leading causes of death.
Conclusions:
- PM/DM continues to significantly impact patient quality of life and survival.
- Older age, visceral involvement (lung, cardiac), dysphagia, cancer, and specific myositis-specific antibodies predict a worse prognosis.
- Further research is needed to improve long-term management and outcomes for PM/DM.
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