Overview: Cardiovascular manifestations and management

Abstract

Systemic sclerosis (scleroderma) has traditionally been considered a disease of tissue fibrosis, but it is now recognized that vascular disease is playing a fundamental role in pathogenesis and the clinical burden. In fact, the primary target in both initiating and propagating the disease may be blood vessels. The tissue injury and fibrosis clearly associates with an autoimmune process and a widespread obliterative vasculopathy of peripheral arteries and microcirculation. The clinical consequences of the vascular insult are evident from the earliest manifestation of the disease: Raynaud's phenomenon, caused by a vascular insult to both nutritional and thermoregulatory vessel of the skin. This unique vascular disease is not limited to the skin but involves all the organs that are involved in scleroderma, including the heart, lungs, kidney, and gastrointestinal tract. Indeed, major life-threatening events that occur in scleroderma are secondary to vascular disturbances such a scleroderma renal crisis, ischemia-reperfusion injury to the myocardium, and pulmonary arterial hypertension. It is now appreciated that the vascular disease can be subclinical with years before the final clinical outcome is expressed. Early in the course of scleroderma, microvascular disease dominates with cutaneous telangiectasias and nailfold capillary aberrations. Heart dysfunction is seen with evidence of microvascular disease resulting in ischemic events and contraction band necrosis that can be explained by both occlusive vascular disease and intermittent vasospasm (i.e., "intramyocardial Raynaud's phenomenon"). Gastrointestinal dysfunction has been thought to be caused by an initial vascular insult that leads to neurogenic dysfunction and bowel dysmotility. Loss of bowel wall smooth muscle and tissue fibrosis are associated with small-vessel disease. Erectile dysfunction is a common complication of scleroderma, a consequence of tissue fibrosis and scleroderma microvascular disease. Later, the clinical complications parallel a picture of the vascular tree being trimmed upstream to larger vessels with macrovascular events including deep digital ulcerations, digital loss, and severe pulmonary vascular disease.