Diagnosis and management of scleroderma peripheral vascular disease

Peripheral vascular involvement occurs in almost all patients who have systemic sclerosis (SSc), because it is rare for them not to experience Raynaud's phenomenon (episodic color changes of the digits, usually in response to cold exposure or stress). The feet as well as the hands are commonly affected. In primary (idiopathic) Raynaud's phenomenon (PRP), digital vasospasm is entirely reversible and does not progress to tissue injury [1]. However, in patients who have SSc, digital ischemia can result in digital ulcers, digital pitting (which is an American Rheumatism Association criterion for SSc [2]), and sometimes gangrene necessitating amputation. Digital ischemia in SSc can be so severe for many reasons, including abnormalities of neuroendothelial control mechanisms (leading to an imbalance of vasoconstriction over vasodilation), structural abnormalities of the vasculature involving both microvessels and the digital arteries, and intravascular factors, including a procoagulant tendency and oxidative stress [3]. Many of these factors are potentially amenable to therapeutic intervention. A key challenge for clinicians is gauging the severity and extent of peripheral vascular involvement in each patient who has SSc and formulating an appropriate investigation and management plan that may vary throughout the disease course. This article considers the assessment and management of SSc-related peripheral vascular disease and highlights the importance of identifying coexisting proximal artery disease.