Related Experiment Videos
Dermatopolymyositis and other connective tissue diseases: a review of 105 cases
Abstract:
Of 105 cases seen over 12 years with mean 4 years followup, there were 69 with polymyositis (PM) and 36 with dermatomyositis (DM). and in 43 this complicated another connective tissue disease (CTD). Primary PM had onset a decade later than others and most severe myopathy occurred in DM. Earliest symptoms were polyarthritis and Raynaud's phenomenon with frequent sicca syndrome (51%). The less than universal prevalence of elevated muscle enzymes (68%), myopathic electromyography (86%). and abnormal muscle biopsy (78%) emphasizes the need for complete evaluation in all cases. Improvement occurred in 69% overall, including all 23 given no therapy or low dose corticosteroids and 59% of the remainder who received high dose corticosteroids with added cytotoxics in one-quarter. Outcome was worse in older patients and in those where weakness exceeded 4 months before diagnosis. Eight of 19 deaths were due to myositis or its therapy which also caused considerable morbidity. Malignancy in 16 cases was temporally related to myositis in half of these cases.
Insights
This study on polymyositis (PM) and dermatomyositis (DM) found that while most patients improved, outcomes were worse in older individuals and those with delayed diagnosis. Myositis complications and malignancy were also noted.
Area of Science:
- Rheumatology
- Neurology
- Immunology
Background:
- Polymyositis (PM) and dermatomyositis (DM) are idiopathic inflammatory myopathies.
- These conditions can be primary or associated with connective tissue diseases (CTD).
- Understanding their clinical course and outcomes is crucial for patient management.
Purpose of the Study:
- To analyze the clinical characteristics, diagnostic findings, and outcomes of patients with polymyositis and dermatomyositis.
- To identify factors influencing disease severity and treatment response.
- To assess the incidence of complications such as malignancy and CTD overlap.
Main Methods:
- Retrospective review of 105 cases of polymyositis and dermatomyositis over 12 years.
- Data collection included clinical presentation, diagnostic tests (muscle enzymes, EMG, biopsy), treatment, and outcomes.
- Follow-up duration averaged 4 years.
Main Results:
- 69 cases of PM and 36 of DM were identified; 43 had CTD overlap.
- Primary PM onset was later; DM showed the most severe myopathy.
- Elevated muscle enzymes, myopathic EMG, and abnormal muscle biopsy were not universal.
- 69% of patients improved overall; outcomes worsened with delayed diagnosis (>4 months) and in older patients.
- Myositis or its therapy caused 8 of 19 deaths; malignancy occurred in 16 cases.
Conclusions:
- Complete diagnostic evaluation is essential for PM and DM due to variable diagnostic marker prevalence.
- Early diagnosis and treatment are critical for better outcomes in inflammatory myopathies.
- PM and DM management requires careful consideration of age, comorbidities, and potential complications like malignancy.