Systemic sclerosis

[Marquetta]

Systemic sclerosis is a rare, chronic autoimmune rheumatic disorder 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, 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)

Limited systemic sclerosis 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 gastroesophageal reflux disease. This type is progresses slowly and is often complicated by pulmonary hypertension, a condition in which blood pressure in the arteries of the lungs (the pulmonary arteries) is abnormally high.
Diffuse systemic sclerosis often causes skin damage that is widespread throughout the body. People who have this type have Raynaud phenomenon and gastrointestinal problems. This type may progress rapidly. Major complications include interstitial lung diseases, which affect the tissue and space around the air sacs of the lungs (alveoli), and a severe kidney problem called scleroderma renal crisis.
Systemic sclerosis without scleroderma rarely occurs without the skin tightening of scleroderma. However, people have antibodies in the blood characteristic of systemic sclerosis and have the same internal problems.