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Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-27967

Müller-Ladner, U; Distler, O; Ibba-Manneschi, L; Neumann, E; Gay, S (2009). Mechanisms of vascular damage in systemic sclerosis. Autoimmunity, 42(7):587-595.

Accepted Version


Although being classified as autoimmune connective tissue disease, dominant components of the pathophysiology of systemic sclerosis (SSc) consists of mechanisms of vascular damage, which can occur early in the course of the disease. Amongst them are abnormal vasoreactivity, hypoxia, insufficient neoangiogenesis and direct damage of vascular and perivascular cells. They result in a decreased capillary blood flow, and subsequently in clinically overt symptoms such as Raynaud's syndrome and fingertip ulcers. In addition, in active disease vascular pathology can affect various other organs, predominantly the lung, the kidney, the heart but also the gastrointestinal tract. Vascular pathology contributes also significantly to overall morbidity and mortality in SSc patients and reduces life expectancy by at least a decade. Fortunately, molecular biology has revealed a number of underlying pathways on the cellular and subcellular levels, including key factors of the aberrant function of (peri)vascular cells and autoimmune effector cells, the dysregulation of vasoconstrictive molecules and their receptors, the upregulation of intracellular signaling kinases and the altered balance of hypoxia-induced vascular growth factors. This increasing knowledge of vascular pathology in SSc has also resulted in novel therapeutic approaches ranging from endothelin antagonists to application of progenitor cells to counteract this aberrant vascular pathology and to support the repair of the dysfunctional vasculature.

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Rheumatology Clinic and Institute of Physical Medicine
DDC:610 Medicine & health
Deposited On:03 Mar 2010 12:15
Last Modified:28 Nov 2013 02:13
Publisher:Taylor & Francis Group
Publisher DOI:10.1080/08916930903002487
PubMed ID:19863377
Citations:Web of Science®. Times Cited: 25
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Scopus®. Citation Count: 30

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