硬皮病(真菌)
医学
细胞外基质
纤维化
系统性硬皮病
疾病
间质性肺病
自然史
生物标志物
免疫学
全身性疾病
病理
肺
内科学
生物
接种
细胞生物学
生物化学
作者
Kristina E N Clark,Christopher P. Denton
标识
DOI:10.1016/s2665-9913(20)30412-4
摘要
Systemic sclerosis is a prototypic fibrotic disease characterised by accumulation of excessive extracellular matrix in affected tissues, including skin, lung, and walls of blood vessels, that can lead to pulmonary hypertension, scleroderma renal crisis, or digital ischaemia. The constellation of clinical manifestations makes systemic sclerosis one of the most challenging of the immune-mediated rheumatic diseases to treat. One of the hallmarks of the disease is its clinical diversity, and the natural history of progression, improvement, or stability in the skin and lung is increasingly appreciated as a predictor of outcome and disease burden. Serum biomarkers that predict future outcomes are an attractive proposition, and the ability to sample extracellular matrix fragments in the blood or other biological samples has opened up possibilities to develop better ways to stratify cases at an early stage that could be applied in clinical trials or practice. Circulating collagen neo-epitopes and their role in the prediction of fibrosis in patients with systemic sclerosis: a multicentre cohort studyThese data suggest that neo-epitopes of type III and IV collagens are promising biomarkers for the assessment and prediction of extracellular matrix remodelling in systemic sclerosis. They could be used in clinical practice to risk stratify patients at risk of progression of fibrosis. Full-Text PDF
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