Scleroderma: Diagnosis, Treatment, and Prognosis

Scleroderma, an autoimmune disorder, affects connective tissue. It generally involves the formation of fibrosis (scar tissue) in the skin and other organs of the body, which leads to extensive thickening of the areas involved. Scleroderma can affect just one organ, or can be widespread throughout the body when it is most intense. While the exact cause of scleroderma has not yet been identified, researchers do believe that genes and environment play a significant role (Scleroderma, William C. Shiel Jr., MD, FACP, FACR, MedicineNet.com).

Diagnosis of Scleroderma

Scleroderma is classified as limited or diffuse depending on the location and severity of the disease. Signs and symptoms might include, but are not limited to:

• Redness and swelling of the skin
• Pruritus
• Decreased range of motion in the fingers, toes, or jaw
• Raynaud’s phenomenon
• Elevated blood pressure
• Headache
• Fatigue
• Shortness of breath
• Constipation

When someone presents to a physician with these complaints, scleroderma is something that could lead him to investigate for the disease further. Diagnosis starts with a set of blood work, including an ANA, which can detect the levels of inflammation in the body. The anticentromere antibody is present in most cases of limited scleroderma, while the antitopoisomerase I antibody (Anti-Scl 70) is usually found in patients with diffuse scleroderma.

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Another test that helps confirm a diagnosis of scleroderma may include a chest x-ray and lung function tests, as well as a CT scan of the chest to check for signs of fibrosis. A heart catheterization may also be in order to check the pressure of the arteries in the heart and lung (caused by elevated blood pressure). If scleroderma is suspected in either the upper or lower bowel, other tests may be ordered to get a closer look at these areas as well.

Treatment of Scleroderma

Treatment really depends on what area(s) of the body is affected by the disease, as well as treating the specific symptoms that arise from the disease. For instance, an elevation in blood pressure requires blood pressure lowering medications in order to protect the kidneys from failure. Antiinflammatory medications are used to help reduce the amount inflammation in the organs that scleroderma is responsible for, while stronger moisturizers can help control itching.

Raynaud’s phenomenon can sometimes be controlled with non-medicinal methods, such as keeping fingers and toes warm, but may require the help of a mild heart medication to keep arteries clear. Sometimes depression medications are used to help with circulation, which has been found to be beneficial for scleroderma sufferers (MedlinePlus: Raynaud’s Disease; U.S. National Library of Medicine). If Raynaud’s becomes unbearable, there are also surgical procedures that can interrupt nerves that are causing constriction of blood vessels.

Of course, a physician will go over all of the treatment options available on a case-by-case basis, as not every person with scleroderma is treated identical. It is important to report all of your symptoms to your physician to be sure you are receiving the best treatment possible.

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Prognosis of Patients with Scleroderma

There is no question that scleroderma is a potentially fatal disease, if not treated and cared for properly. Prognosis is actually quite good for patients who keep in contact with their doctor, and follow prescribed treatment recommendations. This includes regular blood pressure checks, taking medicines as prescribed, and superior communication between doctor and patient.

The time for organ complications that presents the most risk occurs within the first three years of contracting the disease. As long as this critical time is closely watched, the risk for further complications begins to decrease after this three-year time period.

Since the exact causes of scleroderma are not known, research is continually taking place to investigate this complicated disease. This research includes testing different treatment options for their efficacy, as well as developing more sensitive tests to detect the disease at an even earlier stage than is available now. In time, more will be known about this perplexing disease, but researchers have made incredible breakthroughs already in making patients feel more comfortable about their treatments and outcomes.

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