Systemic sclerosis (SSc) is characterized by widespread vascular changes and progressive fibrosis of the skin and internal organs. Alterations of microvessels, preceded by damage and probably an increased apoptosis of endothelial cells, lead to clinical vascular manifestations such as digital ulcers, scleroderma renal crisis and pulmonary arterial hypertension. Of note, vascular manifestations such as Raynaud’s phenomenon and digital ulcers might occur even before the onset of fibrosis indicating a crucial role of microvascular injury for the overall pathogenesis. With respect to pathophysiology, in SSc, two different types of vascular changes can be observed: 1) a destructive vasculopathy of capillaries with vasodilation and rarefication, and 2) a proliferative vasculopathy of small arteries with intimal thickening.
Despite the high morbidity and mortality of vascular complications in SSc, the majority of the currently available animal models only cover the fibrotic aspects of the human disease.
To illustrate the importance of vasculopathy and its possible link with onset of fibrosis, within this lecture, animal models which display vascular features of SSc will be discussed with respect to 1) individual pathophysiological aspects, 2) identified potential molecular key molecules, and 3) suitability for interventional proof of concept-studies.
Disclosure of Interest None Declared
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