Abstract
Systemic sclerosis (SSc) is a rare disorder of the connective tissue characterized by fibrosis of the skin, skeletal muscles and visceral organs. Additional manifestations include activation of the immune system and vascular injury. SSc causes disability and death as the result of end‐stage organ failure. Two clinical subsets of the SSc are accepted: limited cutaneous SSc (lc‐SSc) and diffuse cutaneous SSc (dc‐SSc). At present, the aetiology and pathogenesis of SSc remain obscure, and consequently, disease outcome is unpredictable. Numerous studies suggest that reactive oxidizing species (ROS) play an important role in the pathogenesis of scleroderma. Over the years, several reports have supported this hypothesis for both lc‐SSc and dc‐SSc, although the specific role of oxidative stress in the pathogenesis of vascular injury and fibrosis remains to be clarified. The aim of the present review was to report and comment the recent findings regarding the involvement and role of oxidative stress in SSc pathogenesis. Biomarkers proving the link between ROS and the main pathological features of SSc have been summarized.
Highlights
Scleroderma is an autoimmune disease characterized by vascular abnormalities that include Raynaud’s phenomenon (RP), pulmonary arterial hypertension, fibrosis of skin and visceral organs
The main difference between these two subsets is the speed of disease progression and the extent and severity of skin and visceral involvement in lc-Systemic sclerosis (SSc); fibrosis is mainly restricted to the hands, arms and face, while dcSSc cases report a higher frequency of heart, lung and kidney involvement
advanced oxidation protein products (AOPP) generated by different oxidation patterns can induce the selective triggering of cells to produce H2O2 or NO, suggesting that these oxidation products may be involved in the generation of different types of reactive oxidizing species (ROS) in SSc patients.[30]
Summary
Scleroderma (systemic sclerosis, SSc) is an autoimmune disease characterized by vascular abnormalities that include Raynaud’s phenomenon (RP), pulmonary arterial hypertension, fibrosis of skin and visceral organs.
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