Systemic sclerosis.
Systemic sclerosis.
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DOI：
10.1016/s0140-6736(22)01692-0
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发表时间：
2023-01-28
期刊：
影响因子：
--
通讯作者：
中科院分区：
文献类型：
--
作者：
系统性硬化症，也称为硬皮病，是一种罕见而复杂的自身免疫性结缔组织疾病。一旦被认为是一种无法治疗和不可预测的疾病，研究进展已经提高了我们对其疾病发病机制和临床表型的理解，并扩大了我们的治疗手段。早期和准确的诊断是必不可少的，而正在进行的努力，风险分层患者在预测器官受累和疾病进展的核心作用。在选择最佳治疗策略、平衡副作用与疗效以及根据患者护理目标定制治疗时，需要采用整体方法。本次研讨会回顾了系统性硬化症的多个临床方面，从疾病的早期阶段开始，重点是及时早期发现器官受累。本次研讨会还根据系统性硬化症的病理特征（如炎症，纤维化和血管病变）总结了管理考虑因素，并强调了未满足的需求和未来研究和发现的机会。
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. Early and accurate diagnosis is essential, while ongoing efforts to risk stratify patients have a central role in predicting both organ involvement and disease progression. A holistic approach is required when choosing the optimal therapeutic strategy, balancing the side-effect profile with efficacy and tailoring the treatment according to the goals of care of the patient. This Seminar reviews the multiple clinical dimensions of systemic sclerosis, beginning at a precursor very early stage of disease, with a focus on timely early detection of organ involvement. This Seminar also summarises management considerations according to the pathological hallmarks of systemic sclerosis (eg, inflammation, fibrosis, and vasculopathy) and highlights unmet needs and opportunities for future research and discovery.
影响因子：
4.7
作者：
McMahan ZH;Tucker AE;Perin J;Volkmann ER;Kulkarni S;Ziessman HA;Pasricha PJ;Wigley FM
通讯作者：
Wigley FM