Vascular complications of scleroderma

Systemic sclerosis (SSc) is a multiorgan disease characterized by injury to vascular wall and extensive damage of themicrovessels. The injury of the vascular wall is characterized by the formation of megacapillaries and avascular areas. The reducedcapillary density leads to clinical manifestations such as digital ulcers. These lesions are extremely painful and lead to substantialfunctional disability. Management of digital ulcers includes non-pharmacologic and pharmacologic modalities. Despite thereduced blood flow and reduced partial oxygen pressure levels, there is paradoxically no evidence for a sufficient angiogenesis inthe skin of patients with SSc. Angiogenesis is strongly disturbed in SSc, as demonstrated by Nailfold Video-Capillaroscopychanges, the damage of the vessels evolves progressively from early to late stages and is characterized by different morphologicalaspects. Almost all patients develop Raynaud's phenomenon which, together with structural vasculopathy, results in ulcerationand critical digital ischemia. Many of the severe internal organ complications of SSc are vascular, including pulmonary arterialhypertension (PAH) and scleroderma renal crisis. Structural vascular damage occurs in many vascular beds and contribute topulmonary, renal, cardiac and gastrointestinal complications. SSc has a high case-specific mortality due to organ-basedcomplications including PAH, lung fibrosis, renal failure and involvement of the gastrointestinal tract.