Systemic Sclerosis

An Update

Uwe-Frithjof Haustein, MD, PhD


Lab Med. 2011;42(9):562-572. 

In This Article


Raynaud's phenomenon, increased vascular wall thickness, vascular occlusion, devascularization, and thickening of the basement membrane are features that were described many years ago. Changes in the nailfold capillaries are 1 of the first signs in SSc.[31,32] Furthermore, vascular injury is the basis for the major clinical manifestations of SSc, including pulmonary arterial hypertension, myocardial dysfunction, and renal involvement (scleroderma renal crisis). In internal organs, particularly the kidney, arterioles are characterized by intimal proliferation, thinning of the media, and fibrosis of the adventitia, and exhibit accumulation of proteoglycans and collagens[33] that are probably produced by myofibroblasts. In addition, the vascular pathology is associated with altered vascular function, with increased vasospasm, reduced vasodilatory capacity, hypoxia, oxidative stress, and increased adhesiveness of the blood vessels to platelets and lymphocytes.


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