医学
疾病
全身性疾病
系统性硬皮病
间质性肺病
免疫系统
纤维化
硬皮病(真菌)
重症监护医学
多发性硬化
皮肤病科
肺
内科学
病理
免疫学
接种
作者
Christopher P. Denton,Dinesh Khanna
出处
期刊:The Lancet
[Elsevier]
日期:2017-04-14
卷期号:390 (10103): 1685-1699
被引量:1598
标识
DOI:10.1016/s0140-6736(17)30933-9
摘要
Summary
Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic assessment and follow-up. Additionally, treatments for specific complications have emerged and a growing evidence base supports the use of immune suppression for the treatment of skin and lung fibrosis. Some manifestations of the disease, such as scleroderma renal crisis, pulmonary arterial hypertension, digital ulceration, and gastro-oesophageal reflux, are now treatable. However, the burden of non-lethal complications associated with systemic sclerosis is substantial and is likely to become more of a challenge. Here, we review the clinical features of systemic sclerosis and describe the best practice approaches for its management. Furthermore, we identify future areas for development.
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