Systemic sclerosis: Current concepts of skin and systemic manifestations
Systemic sclerosis: Current concepts of skin and systemic manifestations
复制标题
DOI：
10.1016/j.clindermatol.2018.04.004
复制
发表时间：
2018-07-01
影响因子：
2.7
通讯作者：
Pappas-Taffer, Lisa
中科院分区：
文献类型：
--
作者：
Pearson, David R.;Werth, Victoria P.;Pappas-Taffer, Lisa
系统性硬化症是一种罕见的自身免疫性结缔组织病，多器官系统受累，发病率和死亡率显著相关。皮肤体征和临床表现特别重要，因为它们可以在全身表现之前被识别，从而允许早期风险分层为局限性和弥漫性皮肤亚型，以及早期开始治疗。重要的皮肤表现包括雷诺氏现象、指端溃疡、皮肤硬化、皮肤钙质沉着、毛细血管扩张、瘙痒和色素异常。尽管研究了各种各样的治疗方法，但没有FDA批准的系统性硬化症的药物治疗方法，并且来自高质量研究的数据有限。在下面的综述中，我们将讨论皮肤导向疗法。尽管有证据支持雷诺现象、指端溃疡和皮肤硬化的特定治疗，但评价其他皮肤体征和临床表现治疗的严格研究有限。需要额外的随机对照试验和大型观察性研究来开发未来的循证治疗方案。爱思唯尔公司出版
Systemic sclerosis is an uncommon autoimmune connective tissue disease with multiorgan system involvement and significant associated morbidity and mortality. Cutaneous signs and clinical manifestations are of particular importance, as they may be recognized before systemic manifestations, allowing earlier risk stratification into the limited and diffuse cutaneous subtypes, as well as earlier initiation of treatment. Important cutaneous manifestations include Raynaud's phenomenon, digital ulcers, cutaneous sclerosis, calcinosis cutis, telangiectasias, pruritus, and dyspigmentation. Despite investigation of a wide variety of treatments, no FDA-approved pharmacologic therapies exist for systemic sclerosis, and data from high-quality studies are limited. In the following review, we will discuss skin-directed therapies. Although there is evidence to support specific treatments for Raynaud's phenomenon, digital ulcers, and cutaneous sclerosis, there are limited rigorous studies evaluating the treatment of other cutaneous signs and clinical manifestations. Additional randomized-controlled trials and large observational studies are necessary to develop future evidence-based treatment options. Published by Elsevier Inc.