Scleroderma

Scleroderma is a long-lasting connective tissue disease that is considered an autoimmune disease. Hardening of the skin is one of the most visible signs of the disease.

 

Types of Scleroderma

 

Localized scleroderma appears in limited areas and rarely spreads. It is usually mild and typically does not affect the internal organs.

  • Morphea is waxy patches on the skin of varying sizes, shapes and color. The skin under the patches may thicken and the patches may enlarge or shrink and often may disappear suddenly. Morphea usually appears between the ages of 20 and 50 but is often seen in young children.
  • Linear scleroderma often starts as a streak or line of hardened, waxy skin on an arm, leg or on the forehead. It tends to involve deeper layers of the skin and can affect the motion of the joints. Linear scleroderma usually develops in childhood.

Systemic scleroderma affects the connective tissue in many parts of the body and can involve the skin, esophagus, 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. 

  • Limited scleroderma, sometimes called CREST syndrome, affects the skin of the face, hands and feet and develops slowly over years.
  • Diffuse scleroderma causes the skin thickening to occur quicker and involves more skin areas than in limited disease. In addition, people with diffuse scleroderma have a higher risk of developing “sclerosis” or a tough hardening of the internal organs. Scleroderma can develop in every age group from infants to the elderly, but its onset is most frequent between the ages of 25 to 55.

How Serious is Scleroderma?


Any chronic disease can be serious. The symptoms of scleroderma are very different for each person and can range from very mild to life threatening. The seriousness will depend on the parts of the body affected and how they are affected. A mild case can become more serious if not properly treated. Proper diagnosis and treatment may minimize the symptoms of scleroderma and lessen the chance for permanent damage.

 

How is Scleroderma Diagnosed?


After a physical exam, your doctor may suggest blood tests to check for raised levels of certain antibodies produced by your immune system.  A small sample of your affected skin may also be taken so that it can be examined in the laboratory.

 

Sometimes other blood tests, imaging or organ-function tests are done to help determine whether your digestive system, heart or lungs are affected.

 

How is Scleroderma Treated?


There is treatment available to make scleroderma symptoms feel better as well as medications to help prevent complications like sores or organ damage. Your physician will work with you to identify the treatments that are best for you.

 

Advances in Diffuse Scleroderma


UW Health is now offering a promising new treatment for patients with diffuse scleroderma. Patients with a diagnosis of not more than 5 years who have active interstitial lung disease may be eligible for stem cell transplantation, which has shown significantly more long-term benefits than medication.

 

Patients are not eligible for stem cell transplant if they:

  • Have severe impairment of lung, heart, kidney or liver function; or
  • Have pulmonary arterial hypertension

What Happens to the Patient who Undergoes a Stem Cell Transplant?

 

Patients undergo chemotherapy and total body radiation to destroy the bone marrow. The patient then is infused with his or her own blood-forming stem cells to rebuild the marrow and immune system. This process can eliminate cells that were believed to cause damage to the patient.

 

To learn more or find out if you are eligible:

 

Please contact UW Health Bone Marrow Transplant Clinic Manager Bethaney Campbell, RN, at (608) 262-0455 or by email at bcampbell2@uwhealth.org.