Systemic sclerosis (scleroderma) is a chronic autoimmune disease characterized by widespread damage to small blood vessels, and progressive fibrosis. Systemic sclerosis is more common in females (3:1) and has a peak incidence in 50-60-year olds. Like lupus, patients have aberrant immune system activation resulting in the production of autoantibodies including anti-nuclear antibodies. Microvascular damage occurs early in the disease, particularly in the arterioles of the digits and the kidney (where it causes hypertension and renal failure). Progressive fibrosis occurs later affecting multiple organs including the skin, esophagus, heart and lungs. Damage to the digits (sclerodactyly) and face (taught shiny skin, drawn pursed lips, and atrophy of facial muscles) are most apparent. Angiotensin-converting enzyme (ACE) inhibitors have proven remarkable effective at preventing renal failure in systemic sclerosis patients. In contrast, pulmonary hypertension and progressive pulmonary fibrosis remain as an often fatal complication of this disease. morphea (localized scleroderma) is a form of the disease that is limited to the fingers, forearms and face.