Scleroderma treatment

As an autoimmune disease of the connective tissue (fibers that provide the framework and support for the body), scleroderma involves the tightening and hardening of the skin in certain areas. It is classified as an autoimmune disease because a certain trigger will activate the immune system to cause injury to tissues that result in an injury similar to scar tissue formation. All cases of scleroderma are different, so identifying the type of scleroderma, the stage of the disease, and the organs affected is the key to finding the correct treatment. The four main features of the disease—inflammation, autoimmunity, vascular disease, and tissue fibrosis—are focused on in the treatment.

Types of Scleroderma
There are two main types of scleroderma: diffuse and limited. The diffuse form involves systemic thickening of skin on the extremities, face, and trunk that can quickly progress to hardening after an inflammation phase. Organ involvement—esophagus, bowels, lungs, heart, and kidneys—and destruction can occur early on in this type. The limited form of scleroderma is “limited” to the skin of the fingers and face. The symptoms occur more slowly than in the other form of the disease. This form of the disease is summed up in the word CREST: Calcinosis (formation of tiny calcium deposits in the skin), Raynaud’s phenomenon (spasm of the tiny artery vessels supplying the blood to the fingers, toes, nose, tongue, and ears), Esophagus disease (poor functioning of the muscle in the lower two-thirds of the esophagus), Sclerodactyly (localized thickening and tightness of the skin on the fingers and toes), and Telangiectasias (tiny red areas on the face, hands, mouth, and lips). It is possible for an individual to have only some of these symptoms, and it is also possible that an individual will show signs of both diffuse and limited scleroderma.

Treatment of Scleroderma
As mentioned above, since scleroderma presents differently in every individual, treatment involves fixing the direct problem. Many doctors may be involved with treatment, specializing in different areas; a dermatologist will be used for skin problems, a nephrologist will deal with the kidneys, a cardiologist will help with the heart, a gastroenterologist will specialize in intestinal problems, and a pulmonary specialist will help with the lungs.

Common Medications
Anti-inflammatory medications are used in most cases. Inflammation that causes arthritis is treated with over-the-counter anti-inflammatory drugs (known as NSAIDs), such as ibuprofen, or steroids, such as prednisone. Exercising regularly can also help with arthritic inflammation. The other type of inflammation treated is related to the injury of the skin and other tissues due to the stages of the disease, and this type doesn’t respond to NSAIDs.

Immunosuppressive Therapy
The inflammatory phase of scleroderma, since it doesn’t respond to over-the-counter NSAIDs, is sometimes treated with immunosuppressive therapy. The idea: an autoimmune process is causing the inflammation and the negative result is tissue damage and fibrosis. Immunosuppressing drugs include: methotrexate, cyclosporine, antithymocyte globulin, mycophenolate mofetil, and cyclophosphamide. The most effective drugs are the last three.

Drug Therapy of Vascular Disease
Raynaud’s phenomenon can be treated on its own. Fingers and toes will often turn blue and be very cold, mostly in response to generally cold temperatures or emotional upset. Do the following: don’t smoke because this will further narrow the blood vessels; dress warmly, especially on the hands and feet; use relaxation techniques, such as meditation; or talk to your doctor about using certain drugs if the problem is really bad. Also, vessel dilating medications may be needed to prevent lung and kidney problems.

Other Therapy
Occupational or physical therapy may benefit some individuals. Therapists can help you manage pain, improve strength and mobility, and help you learn how to perform daily tasks more independently.

In some cases, surgery may be necessary. Amputation of a limb, such as a finger that has had too many ulcers, is sometimes necessary. Also, a lung transplant may be needed in those who have high blood pressure in their arteries to the lungs.

Last updated on Mar 10th, 2011 and filed under Musculoskeletal Disorders. Both comments and pings are currently closed.

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