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Oxidative stress and the pathogenesis of scleroderma: the Murrell's hypothesis revisited
- Publication Year :
- 2008
-
Abstract
- Systemic sclerosis (SSc, scleroderma) is a devastating, immune-mediated, multisystem disorder characterized by microvasculature damage, circulating autoantibodies, and fibroblast activation, leading to massive fibrosis of skin, vessels, muscles, and visceral organs. Scleroderma causes disability and death as the result of end-stage organ failure. At present, no specific diagnostic nor therapeutic tools are available to handle the disease. In spite of significant effort, the etiology and pathogenesis of SSc remain obscure and, consequently, the disease outcome is unpredictable. Several years ago, Murrell suggested a unifying hypothesis linking the pathogenesis of scleroderma to the generation of a large excess of reactive oxygen species. This hypothesis has been substantiated by several reports indicating the presence of an abnormal redox state in patients with scleroderma. This review will summarize the available evidence supporting the link between free radicals and the main pathological features of scleroderma.
- Subjects :
- EXHALED NITRIC-OXIDE
Systemic disease
Pathology
medicine.medical_specialty
GROWTH-FACTOR
SMOOTH-MUSCLE-CELLS
Immunology
Disease
medicine.disease_cause
Autoantigens
Scleroderma
Pathogenesis
HUMAN ENDOTHELIAL-CELLS
Fibrosis
Humans
Immunology and Allergy
Medicine
Receptors, Platelet-Derived Growth Factor
skin and connective tissue diseases
Autoantibodies
Autoimmune disease
Scleroderma, Systemic
RAYNAUDS-PHENOMENON SECONDARY
integumentary system
business.industry
medicine.disease
Connective tissue disease
HUMAN LUNG FIBROBLASTS
Oxidative Stress
ANGIOTENSIN-II
SUPEROXIDE ANION PRODUCTION
REACTIVE OXYGEN
Reactive Oxygen Species
business
SYSTEMIC-SCLEROSIS PATIENTS
Oxidative stress
Signal Transduction
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Accession number :
- edsair.doi.dedup.....d6cc85a4a83025e5e5a147044598bc52