Micro- and macrovascular abnormalities in systemic sclerosis

Systemic sclerosis is a connective tissue disorder characterized by fibrosis of the skin, joints and internal organs. It is also characterized by microvascular abnormalities, like Raynaud’s phenomenon, present in more than 95% of the patients, and affects the quality of life.



Treatment with bosentan, an endothelin-1 antagonist, during a period of 16 weeks, in an open, uncontrolled study, resulted in a subjective improvement in Raynaud’s phenomenon. We were not able to demonstrate an objective improvement in blood flow, capillary permeability, number of nailfold capillaries and other parameters of the microcirculation



Changes in the microcirculation in patients with systemic sclerosis are due to the disease itself and not due to traditional cardiovascular risc factors. Local capillary permeability of the nailfolds is increased in patients with systemic sclerosis. We found no evidence for increased capillary permeability to be a generalized phenomenon, although we did find a decreased capillary permeability and a decreased capillry density at the level of the ankle.



In contrast with other auto-immune rheumatic diseases no increased prevalence of clinical manifest or early subclinical atherosclerosis was found. We also could not demonstrate increased accumulation of advanced glycation endproducts, present in diseases like atherosclerosis and diabetes mellitus.



In summary, systemic sclerosis is characterized by microvascular abnormalities. We need more adequate therapy to treat symptoms of and to prevent microvascular abnormalities.

	Title and contents
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	Chapter 5
	Chapter 6
	Chapter 7
	Chapter 8
	Summary
	Samenvatting
	Dankwoord
	Complete thesis

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