Systemic Scleroderma (systemic sclerosis)

The changes occurring in systemic scleroderma may affect the connective tissue in many parts of the body. Systemic scleroderma can involve the skin, esophagus, gastrointestinal tract (stomach and bowels), lungs, kidneys, heart, and other internal organs. It can also affect blood vessels, muscles, and joints. The tissues of involved organs become hard and fibrous, causing them to function less efficiently. The term systemic sclerosis indicates that “sclerosis” (hardening) may occur in the internal systems of the body. There are two major recognized patterns that the illness can take, diffuse or limited disease. In diffuse scleroderma, skin thickening occurs more rapidly and involves more skin areas than in limited disease. In addition, people with diffuse scleroderma have a higher risk of developing “sclerosis” or fibrous hardening of the internal organs.

About one-half of patients have a slower and more benign illness called limited scleroderma. In limited scleroderma, skin thickening is less widespread, typically confined to the fingers, hands, and face, and develops slowly over years.

Although internal problems occur, they are less frequent and tend to be less severe than in diffuse scleroderma, and are usually delayed in onset for many years. However, persons with limited scleroderma can develop pulmonary hypertension, a condition in which the blood vessels of the lungs become narrow, leading to impaired blood flow through the lungs.

Limited scleroderma is sometimes called the CREST syndrome. CREST stands for the initial letters of five common features:

Calcinosis; Raynaud’s phenomenon; Esophageal dysfunction; Sclerodactyly; and Telangiectasia (these features are discussed later in this booklet). To further complicate the terminology, some people with diffuse disease will go on to develop calcinosis and telangiectasias so that they also have the features of CREST.

Although most patients can be classified as having either diffuse or limited disease, different people may have different symptoms and different combination of symptoms of the illness.