Scleroderma and Primary Myocardial Disease

Excerpt

Systemic sclerosis is a rare, complex, and chronic multisystem disease. Scleroderma means hard and thick skin. It is derived from the ancient Greek words "skleros," which means (hard) and "derma," which means (skin). Skin involvement is one of the features of systemic sclerosis (SSc). The hallmark features of systemic sclerosis include immune dysregulation with inflammation, vascular injury with an anatomic and functional abnormality, and systemic interstitial fibrosis of organs and skin, leading to multisystem organ damage. Cardiac involvement is well recognized in systemic sclerosis, which can affect the pericardium, the myocardium, and the conduction system. Primary cardiac disease is reported in both diffuse and limited systemic sclerosis. However, it was found to be more prevalent and more severe in diffuse systemic sclerosis.