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Long-term outcome in polymyositis and dermatomyositis
I M Bronner1, M F G van der Meulen, M de Visser
1Department of Neurology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
Annals of the Rheumatic Diseases
|April 12, 2006
Summary
Polymyositis and dermatomyositis are serious, treatable diseases with at least 10% mortality. Long-term outcomes show significant disability and reduced quality of life, despite regained muscle strength in many patients.
Area of Science:
- Rheumatology
- Immunology
- Neurology
Background:
- Polymyositis and dermatomyositis are considered treatable, but long-term prognosis and prognostic factors remain poorly understood.
- Existing literature shows wide variations in long-term outcomes and prognostic indicators for these inflammatory myopathies.
Purpose of the Study:
- To analyze prognostic outcome factors in polymyositis and adult dermatomyositis.
- To identify predictors of mortality, clinical outcomes, and disease course in patients with inflammatory myopathies.
Main Methods:
- Determined mortality rates, clinical outcomes (muscle strength, disability, drug use, quality of life), and disease course.
- Analyzed prognostic factors influencing these outcomes in a cohort of 110 patients with a median follow-up of 5 years.
Main Results:
- Disease-related mortality occurred in at least 10% of patients, primarily due to associated cancer and pulmonary complications.
- 80% of patients experienced a polycyclic or chronic continuous disease course; only 20% achieved remission off drugs.
- Muscle weakness was linked to older age (OR 3.6), and disability to male sex (OR 3.1). Jo-1 antibodies predicted persistent drug use (OR 4.4).
Conclusions:
- Inflammatory myopathies like dermatomyositis and polymyositis represent serious conditions with significant disease-related mortality.
- These diseases substantially impact long-term disability and quality of life, even when muscle strength improves.
- Prognostic factors such as age, sex, and specific antibodies (Jo-1) influence patient outcomes and disease management.