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Muscle involvement in the scleroderma syndromes.
R A Ringel1, J E Brick, J F Brick
1Department of Neurology, West Virginia University Health Sciences Center, Morgantown 26506.
Archives of Internal Medicine
|December 1, 1990
Summary
Muscle involvement in scleroderma can mimic inflammatory myopathies, presenting as symmetrical proximal weakness. Diagnosis relies on creatine kinase levels, electromyography, and muscle histopathology revealing vasculopathy or polymyositis.
Area of Science:
- Rheumatology
- Neurology
- Pathology
Background:
- Scleroderma and connective tissue diseases can manifest with muscle involvement.
- Muscle weakness in these conditions can be indistinguishable from other inflammatory myopathies.
Purpose of the Study:
- To investigate the characteristics of muscle involvement in patients with scleroderma or related overlap syndromes.
- To determine the diagnostic utility of creatine kinase, electromyography, and muscle histopathology.
Main Methods:
- Retrospective analysis of 14 patients with scleroderma or overlap syndromes.
- Assessment of clinical presentation, creatine kinase levels, electromyography findings, and muscle histopathology.
- Histopathological examination focused on identifying vasculopathy and polymyositis.
Main Results:
- Muscle involvement presented as symmetrical proximal weakness in all patients.
- Creatine kinase levels and electromyography were effective in demonstrating muscle involvement.
- Muscle histopathology revealed scleroderma vasculopathy in some patients and polymyositis in others.
- These findings often occurred independently of specific scleroderma features or overlap syndromes.
Conclusions:
- Muscle involvement in scleroderma is clinically similar to other inflammatory myopathies.
- Diagnostic tools like creatine kinase, electromyography, and muscle biopsy are crucial for identification.
- Scleroderma vasculopathy and polymyositis are key histopathological findings, sometimes occurring independently of specific systemic features.