What Factors Affect a Scleroderma Prognosis?

L. Whitaker

Scleroderma prognosis is affected by a wide variety of medical factors. In general, scleroderma prognosis depends on the individual's overall state of health and the monitoring of complicating factors such as high blood pressure. Those with severe skin symptoms at the onset of the disease tend to have a lower survival rate. The greatest risk of organ complications is believed to occur within the first three years after the initial appearance of skin symptoms. Approximately 66 percent of those diagnosed with scleroderma live for at least a decade.

Swollen blood vessels may be symptom of the autoimmune condition scleroderma.
Swollen blood vessels may be symptom of the autoimmune condition scleroderma.

Scleroderma is an autoimmune disorder involving the connective tissues of the body, including the skin. Localized scleroderma is limited to one or two areas of the body, typically the hands and face. Systemic scleroderma, also known as diffuse scleroderma or systemic sclerosis, is spread over many organs and areas of the skin.

A patient's scleroderma prognosis depends on her or his overall state of health.
A patient's scleroderma prognosis depends on her or his overall state of health.

Scleroderma can cause damage to kidneys, lungs, the heart, or the gastrointestinal system. Inflammation and scarring caused by scleroderma can result in severe joint contraction. Scarring in the lungs could lead to increased blood pressure. Systemic scleroderma is sometimes associated with abnormal heart rhythms or heart failure, and symptoms can sometimes overlap with those of other autoimmune disorders, such as lupus or polymyositis. Systemic scleroderma prognosis could rapidly become worse, leading to premature death.

Localized scleroderma is sometimes called CREST scleroderma, referring to the first letters of classic symptoms of this disorder. Calcinosis describes calcium deposits that cause hard areas on the skin of the knees, fingers, or elbows. Raynaud's phenomenon is the name for an arterial spasm that causes the skin to turn blue, white, and red when exposed to extreme temperatures or emotional upset. Esophageal dysfunction results from scarring in the esophagus, while sclerodactyly limits the range of function in the fingers and toes due to skin thickening and tightening. The final symptom in the CREST group is telangiectasias, which is the presence of tiny red areas on the hands and face or in the mouth that are caused by dilated capillaries.

Typical symptoms can include thickening of the skin, scarring known as fibrosis, inflammation, and overactive immune response. Localized scleroderma prognosis generally includes the expectation of a normal lifespan but with the likelihood of medical complications due to eventual organ involvement. Sometimes scleroderma can be localized without complications for many years before there is risk of internal organ damage. An individual's quality of life can be affected by a scleroderma prognosis that predicts chronic pain, limited mobility, and changes in appearance that affect self-esteem.

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