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Systemic sclerosis, also known as scleroderma, is a rare and complex autoimmune connective-tissue disease. Once considered an untreatable and unpredictable condition, research advancements have improved our understanding of its disease pathogenesis and clinical phenotypes and expanded our treatment armamentarium.
E. Volkmann, K. Andréasson, V. Smith
semanticscholar +5 more sources
Systemic sclerosis (scleroderma) is a rare generalized disorder of connective tissue origin. This condition is predominantly a clinical diagnosis, based on the clinical signs and symptoms.
Ahathya R, Deepalakshmi D, Emmadi Pamela
doaj +3 more sources
Treatment of systemic sclerosis. [PDF]
Proper classification of patients into diffuse cutaneous and limited cutaneous subsets and the anticipation of complications are the keys to the management of individuals with systemic sclerosis (scleroderma). Patients with early diffuse disease and rapidly progressive skin thickening are at highest risk to develop serious internal organ involvement ...
Thomas A. Medsger
+15 more sources
Systemic sclerosis is a complex autoimmune disease characterized by a chronic and frequently progressive course and by extensive patient-to-patient variability. Like other autoimmune diseases, systemic sclerosis occurs more frequently in women, with a peak of onset in the fifth decade of life.
Yannick, Allanore+6 more
openaire +4 more sources
Myofibroblast transcriptome indicates SFRP2hi fibroblast progenitors in systemic sclerosis skin
Skin and lung fibrosis in systemic sclerosis (SSc) is driven by myofibroblasts, alpha-smooth muscle actin expressing cells. The number of myofibroblasts in SSc skin correlates with the modified Rodnan skin score, the most widely used clinical measure of ...
T. Tabib+11 more
semanticscholar +1 more source
Tocilizumab Prevents Progression of Early Systemic Sclerosis–Associated Interstitial Lung Disease
Tocilizumab (TCZ) has demonstrated lung function preservation in 2 randomized controlled trials in early systemic sclerosis (SSc). This effect has yet to be characterized in terms of radiographically evident quantitative lung involvement.
D. Roofeh+7 more
semanticscholar +1 more source
The burden of systemic sclerosis in Switzerland – the Swiss systemic sclerosis EUSTAR cohort
OBJECTIVES Characteristics of Swiss patients with systemic sclerosis have not been described so far. The aim of the current study was to identify unmet needs in comparison with other European countries that could inform specific interventions
Jasmin Hernández+23 more
doaj +1 more source
Predictors of fatigue severity in early systemic sclerosis: a prospective longitudinal study of the GENISOS cohort. [PDF]
ObjectivesLongitudinal studies examining the baseline predictors of fatigue in SSc have not been reported. Our objectives were to examine the course of fatigue severity over time and to identify baseline clinical, demographic, and psychosocial predictors
Assassi, Shervin+10 more
core +19 more sources
Current Concepts on the Pathogenesis of Systemic Sclerosis
From the clinical standpoint, systemic sclerosis (SSc) is characterized by skin and internal organ fibrosis, diffuse fibroproliferative vascular modifications, and autoimmunity. Clinical presentation and course are highly heterogenous and life expectancy
M. Truchetet+2 more
semanticscholar +1 more source
Oxidative stress-dependent activation of collagen synthesis is induced in human pulmonary smooth muscle cells by sera from patients with scleroderma-associated pulmonary hypertension [PDF]
Pulmonary arterial hypertension is a major complication of systemic sclerosis. Although oxidative stress, intima hyperplasia and a progressive vessel occlusion appear to be clearly involved, the fine molecular mechanisms underpinning the onset and ...
Boin, Francesco+8 more
core +8 more sources