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Ultrastructure of systemic sclerosis inflammatory myopathy
H J Finol1, A Marquez, H Rivera
1Center of Electron Microscopy, Sciences Faculty, Central University of Venezuela, Caracas.
Journal of Submicroscopic Cytology and Pathology
|April 1, 1994
Summary
Systemic sclerosis (SS) can cause inflammatory myopathy, a distinct muscle disease. Ultrastructural study of muscle biopsies revealed characteristic fiber atrophy, necrosis, and capillary abnormalities in patients with SS.
Area of Science:
- Rheumatology
- Neurology
- Pathology
Background:
- Systemic sclerosis (SS) is a multisystem autoimmune disease.
- Muscle involvement in SS can lead to proximal muscular weakness.
- The specific nature of myopathy in SS requires detailed investigation.
Purpose of the Study:
- To ultrastructurally characterize muscle pathology in patients with systemic sclerosis and proximal muscular weakness.
- To identify distinct pathological features of myopathy associated with systemic sclerosis.
- To differentiate SS-associated myopathy from other muscle diseases.
Main Methods:
- Ultrastructural analysis of muscle biopsies from 7 patients with systemic sclerosis and proximal weakness.
- Detailed examination of muscle fiber morphology, connective tissue, and inflammatory infiltrates.
- Assessment of capillary structure, including endothelial cells and basement membranes.
Main Results:
- Consistent findings of inflammatory myopathy, including fiber atrophy, occasional necrosis, and connective tissue proliferation.
- Presence of specific ultrastructural findings: filamentous bodies, concentric laminated bodies, and mononuclear cell infiltration (lymphocytes, macrophages, mast cells).
- Significant capillary abnormalities observed: endothelial alterations, basement membrane thickening/reduplication, and cylindric confronting cisternae.
Conclusions:
- Inflammatory myopathy in systemic sclerosis exhibits distinct pathological features.
- The observed ultrastructural changes suggest a unique clinicopathological entity.
- This study supports the classification of SS-associated inflammatory myopathy as a distinct condition.