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[Systemic sclerosis: pathophysiology of a multifaceted disease].
- Source :
-
Presse medicale (Paris, France : 1983) [Presse Med] 2006 Dec; Vol. 35 (12 Pt 2), pp. 1903-15. - Publication Year :
- 2006
-
Abstract
- Systemic sclerosis is a rare disease characterized by vascular hyperreactivity and collagen deposition. Endothelial cell, fibroblast and lymphocyte abnormalities have been reported in systemic sclerosis. Fibroblast dysfunction is characterized by uncontrolled activation of the transforming growth factor-beta (TGF-beta) pathway and excess synthesis of both connective tissue growth factor (CTGF) and free radicals. These promote the accumulation of extracellular matrix. Endothelial cells produce excess quantities of endothelin 1 and inducible NO synthase. They also undergo early apoptosis. Oxidative stress appears to play a major role in disease progression. Increased levels of interleukin 4, a profibrotic cytokine, have been detected in plasma and skin of systemic sclerosis patients. Autoantibodies are detectable in the serum of almost all systemic sclerosis patients. Some are directed against well-identified ubiquitous nuclear proteins and have no demonstrated pathogenic role. Other autoantibodies bind to endothelial cells or fibroblasts and may have a pathogenic role.
- Subjects :
- Adult
Animals
Apoptosis
Autoantibodies immunology
Cells, Cultured
Disease Models, Animal
Disease Progression
Endothelium, Vascular physiopathology
Female
Fibroblasts metabolism
Free Radicals
Humans
Interleukin-4 metabolism
Male
Mice
Mice, Inbred BALB C
Oxidative Stress
Phenotype
Pregnancy
Scleroderma, Systemic blood
Scleroderma, Systemic etiology
Scleroderma, Systemic genetics
Scleroderma, Systemic immunology
Scleroderma, Systemic metabolism
Scleroderma, Systemic pathology
Skin metabolism
Transforming Growth Factor beta physiology
Scleroderma, Systemic physiopathology
Subjects
Details
- Language :
- French
- ISSN :
- 0755-4982
- Volume :
- 35
- Issue :
- 12 Pt 2
- Database :
- MEDLINE
- Journal :
- Presse medicale (Paris, France : 1983)
- Publication Type :
- Academic Journal
- Accession number :
- 17159716
- Full Text :
- https://doi.org/10.1016/s0755-4982(06)74924-7