Overview of pathogenesis of systemic sclerosis

被引：176

作者：

Abraham, D. J. ^{[1
]}

Krieg, T. ^{[2
]}

Distler, J. ^{[3
,4
]}

Distler, O. ^{[5
,6
]}

机构：

[1] UCL, Royal Free & Univ Coll Med Sch, Ctr Rheumatol & Connect Tissue Dis, London NW3 2PF, England

[2] Univ Cologne, Dept Dermatol, D-5000 Cologne, Germany

[3] Univ Erlangen Nurnberg, Dept Internal Med 3, Erlangen, Germany

[4] Univ Erlangen Nurnberg, Inst Clin Immunol, Erlangen, Germany

[5] Univ Zurich, Rheumaklin, Zurich, Switzerland

[6] Univ Zurich, Inst Med Phys, Zurich, Switzerland

来源：

RHEUMATOLOGY | 2009年 / 48卷

关键词：

Endothelial cell; Endothelin-1; Vasculopathy; Fibrosis; Scleroderma; ANTIENDOTHELIAL CELL ANTIBODIES; ENDOTHELIAL GROWTH-FACTOR; GENE-EXPRESSION; SCLERODERMA FIBROBLASTS; MICROVASCULAR PERICYTES; MESENCHYMAL TRANSITION; PULMONARY-HYPERTENSION; EXTRACELLULAR-MATRIX; SMOOTH-MUSCLE; SKIN;

D O I：

10.1093/rheumatology/ken481

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

The aetiology of SSc is subject to ongoing research, as the precise events that underlie the development of this disease remain unclear. The pathogenesis is known to involve endothelium, epithelium, fibroblasts, innate and adaptive immune systems and their component immunological mediators. Endothelial cell damage may be the initiating factor, but the precise triggering event(s) remain elusive. Angiogenesis also appears to be dysregulated. Vasculopathy shows similarities in different organs (e.g. pulmonary arterial hypertension, renal disease, digital tip ulcers). Endothelin-1 is a potent mediator of vasculopathy, and hence represents a highly relevant target for intervention of vascular features in SSc.

引用

页码：3 / 7

页数：5

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