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系统性硬化症发病机制与治疗的最新进展

Recent advances on pathogenesis and therapies in systemic sclerosis.

作者信息

Yazawa Norihito, Fujimoto Manabu, Tamaki Kunihiko

机构信息

Department of Dermatology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.

出版信息

Clin Rev Allergy Immunol. 2007 Oct;33(1-2):107-12. doi: 10.1007/s12016-007-8009-2.

DOI:10.1007/s12016-007-8009-2
PMID:18064573
Abstract

Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by extensive fibrotic changes in various organs, including skin and lung. Although the etiology of SSc remains unknown, three major abnormalities, abnormal humoral immunity, microvasculature, and fibroblast dysfunctions are considered to play important roles. Significant progress has been made in understanding the pathogenesis on SSc, and has been also providing clues to the treatment for this disease. This review summarizes recent advances on the pathogenesis and new therapeutic strategy for SSc.

摘要

系统性硬化症(SSc)是一种全身性自身免疫性疾病,其特征是包括皮肤和肺在内的各种器官发生广泛的纤维化改变。尽管SSc的病因尚不清楚,但三种主要异常,即异常的体液免疫、微血管病变和成纤维细胞功能障碍被认为起着重要作用。在理解SSc的发病机制方面已经取得了重大进展,也为该疾病的治疗提供了线索。本综述总结了SSc发病机制和新治疗策略的最新进展。

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Recent advances on pathogenesis and therapies in systemic sclerosis.系统性硬化症发病机制与治疗的最新进展
Clin Rev Allergy Immunol. 2007 Oct;33(1-2):107-12. doi: 10.1007/s12016-007-8009-2.
2
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3
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引用本文的文献

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Reumatologia. 2017;55(4):163-168. doi: 10.5114/reum.2017.69776. Epub 2017 Aug 31.
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T and NK Cell Phenotypic Abnormalities in Systemic Sclerosis: a Cohort Study and a Comprehensive Literature Review.系统性硬化症中T细胞和自然杀伤细胞的表型异常:一项队列研究及综合文献综述
Clin Rev Allergy Immunol. 2015 Dec;49(3):347-69. doi: 10.1007/s12016-015-8505-8.
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Autoantibody-mediated regulation of B cell responses by functional anti-CD22 autoantibodies in patients with systemic sclerosis.

本文引用的文献

1
Stimulatory autoantibodies to the PDGF receptor in systemic sclerosis.系统性硬化症中针对血小板衍生生长因子受体的刺激性自身抗体。
N Engl J Med. 2006 Jun 22;354(25):2667-76. doi: 10.1056/NEJMoa052955.
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Cyclophosphamide versus placebo in scleroderma lung disease.环磷酰胺与安慰剂治疗硬皮病肺病的对比
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3
Magnetic resonance imaging for the detection of myocardial fibrosis in scleroderma.磁共振成像用于检测硬皮病中的心肌纤维化。
自身抗体通过功能性抗 CD22 自身抗体对系统性硬化症患者 B 细胞反应的调节作用。
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Transcriptional regulation of Bim by FOXO3a and Akt mediates scleroderma serum-induced apoptosis in endothelial progenitor cells.FOXO3a和Akt对Bim的转录调控介导了硬皮病血清诱导的内皮祖细胞凋亡。
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Diminished induction of skin fibrosis in mice with MCP-1 deficiency.单核细胞趋化蛋白-1缺乏的小鼠皮肤纤维化诱导减弱。
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5
Endothelin-1 serum levels correlate with MCP-1 but not with homocysteine plasma concentration in patients with systemic sclerosis.在系统性硬化症患者中,内皮素-1血清水平与单核细胞趋化蛋白-1相关,但与同型半胱氨酸血浆浓度无关。
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A pilot trial of treprostinil for the treatment and prevention of digital ulcers in patients with systemic sclerosis.一项关于曲前列尼尔治疗和预防系统性硬化症患者指端溃疡的试点试验。
J Am Acad Dermatol. 2006 May;54(5):880-2. doi: 10.1016/j.jaad.2006.02.004.
7
[Bosentan for treatment of active digital ulcers in patients with systemic sclerosis].波生坦治疗系统性硬化症患者的活动性指端溃疡
Presse Med. 2006 Apr;35(4 Pt 1):587-92. doi: 10.1016/s0755-4982(06)74645-0.
8
Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease.儿童期起病的系统性硬化症:分类、临床及血清学特征,以及与成人起病疾病相比的生存率
J Rheumatol. 2006 May;33(5):1004-13. Epub 2006 Apr 1.
9
DNA topoisomerase I binding to fibroblasts induces monocyte adhesion and activation in the presence of anti-topoisomerase I autoantibodies from systemic sclerosis patients.在系统性硬化症患者抗拓扑异构酶I自身抗体存在的情况下,DNA拓扑异构酶I与成纤维细胞结合会诱导单核细胞黏附和活化。
Arthritis Rheum. 2006 Mar;54(3):963-73. doi: 10.1002/art.21646.
10
Mycophenolate mofetil as first-line treatment improves clinically evident early scleroderma lung disease.霉酚酸酯作为一线治疗可改善临床明显的早期硬皮病肺病。
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