bone, joint, and muscle disorders
Systemic Sclerosis
Introduction
Systemic sclerosis is a rare, chronic Overview of Autoimmune Disorders of Connective Tissue characterized by degenerative changes and scarring in the skin, joints, and internal organs and by blood vessel abnormalities. The cause of systemic sclerosis is unknown. Swelling of the fingers, intermittent coolness and blue discoloration of the fingers, joints freezing in permanent (usually flexed) positions (contractures), and damage to the gastrointestinal system, lungs, heart, or kidneys may develop. People often have antibodies in the blood characteristic of an autoimmune disorder. There is no cure for systemic sclerosis, but symptoms and organ dysfunction can be treated. Systemic sclerosis causes an overproduction of collagen and other proteins in various tissues. The cause of systemic sclerosis is not known. The disorder is 4 times more common among women and most common among people aged 20 to 50. It is rare among children. Symptoms of systemic sclerosis may occur as part of Mixed Connective Tissue Disease (MCTD) , and some people with mixed connective tissue disease ultimately develop severe systemic sclerosis. Systemic sclerosis can be categorized as Limited systemic sclerosis (CREST syndrome) Diffuse systemic sclerosis Systemic sclerosis without scleroderma (systemic sclerosis sine scleroderma) affects just the skin or mainly only certain parts of the skin and is also called CREST syndrome . People who have this type develop skin tightening (scleroderma) over the face, hands, forearms, lower legs, and feet. People may also have often causes skin damage that is widespread throughout the body. People who have this type have Raynaud Syndrome and gastrointestinal problems. This type may progress rapidly. Major complications include Systemic sclerosis without sclerodermararely occurs without the skin tightening of scleroderma. However, people have antibodies in the blood characteristic of systemic sclerosis and have the same internal problems. 