According to the Scleroderma Foundation, scleroderma is a chronic connective tissue disease, and is generally classified as a rheumatic autoimmune disease. In patients with scleroderma, the body over-produces collagen, reacting as if there were an injury needing repair. This over-production of collagen prevents various organs in the body from functioning normally.
While this condition is poorly understood, there is some information about the disease that we do know. Read on to learn 10 facts about scleroderma.
1. Scleroderma is more common than you think
It’s estimated that 300,000 Americans live with scleroderma. Of these 300,000 patients, approximately 1/3 live with the systemic form of the disease. However, it’s possible that the number of scleroderma patients is actually much higher, since diagnosing this skin condition can be difficult, as the disorder bears a lot of similarities to other autoimmune diseases, such as polymyositis.
2. Localized scleroderma is the first main type
The two main types of scleroderma are localized and systemic. With localized scleroderma, symptoms such as skin thickening and collagen overproduction are limited to a few places on the skin or muscles, and internal organs are usually not affected. In general, localized scleroderma is relatively mild and rarely develops into systemic scleroderma.
Morphea and linear scleroderma are two sub-classifications of this form of the disease, which appear as patches or streaks on the skin, respectively.
3. Systemic scleroderma is the second main type
In contrast to localized scleroderma, systemic scleroderma affects the connective tissue in many parts of the body, including the skin, esophagus, gastrointestinal tract (stomach and bowels), lungs, kidneys, heart, and other internal organs. It can even impact blood vessels, muscles, and joints. These tissues become hard and fibrous, which decreases their function.
There are two sub-classifications of systemic scleroderma – diffuse and limited. Diffuse scleroderma results in a rapid skin thickening across a larger portion of the skin. Patients with this form of the disease have more internal organ involvement as well. Conversely, limited scleroderma occurs when the skin thickening is less widespread, and is usually confined to the fingers, hands and face. It tends to develop slowly over time.
4. Limited scleroderma is also called CREST syndrome
Limited scleroderma is sometimes referred to as CREST syndrome, an acronym which stands for the different symptoms this condition causes:
- Calcinosis – an accumulation of calcium deposits under the skin, which may cause pain
- Raynaud’s – a phenomenon in which small arteries that supply blood to the skin constrict excessively in response to cold, limiting blood supply to one’s fingers and toes and changing their color
- Esophageal dysfunction – a stiffening of the gastrointestinal tract muscles, resulting in reflux and indigestion
- Sclerodactyly – the hardening of the skin on one’s fingers and/or toes
- Telangiectasias – round, red spots on the skin’s surface as a result of widened small blood vessels
5. Scleroderma can affect one’s lungs
Pulmonary symptoms can occur in patients with systemic scleroderma. For example, patients may develop pulmonary hypertension, a condition in which the lung’s blood vessels narrow, which results in impaired blood flow in the lungs. This, in turn, causes shortness of breath.
6. Your gender, age, ethnicity and genetics may play a role
Scleroderma affects women up to three to four times more frequently than their male counterparts. According to the Mayo Clinic, the condition most commonly occurs between the ages of 30 and 50, although children can also develop the disease. One’s ethnic background may also influence the risk of developing the disease, the age of onset, and the severity of one’s symptoms. Although it’s believed that genetics play a role in the development of scleroderma, genetic factors are thought to only predispose a person to the disease, rather than cause it.
7. Other autoimmune issues may co-occur
Since scleroderma is an autoimmune disease, it may occur in conjunction with other autoimmune issues. According to the Mayo Clinic, between 15-20% of scleroderma patients have another autoimmune disease, such as rheumatoid arthritis, lupus or Sjogren’s syndrome. This is why it’s important for patients to get evaluated for other potential co-morbidities as well.
8. There is no cure, but treatments do exist
While there is no known cure for scleroderma, treatment options do exist to help patients manage their symptoms and to prevent further complications of the disease. For example, your doctor may prescribe steroids to help you cope with skin symptoms. Blood pressure medications may also be used to treat Raynaud’s phenomenon. Anti-acids and antibiotics can help reduce digestive issues and prevent infections. Immunosuppressants may be prescribed to reduce overactivity of your immune system and to decrease damaging inflammation. And finally, pain medications may also be used to decrease pain if over-the-counter pain medications aren’t effective enough.
9. Surgery may be necessary
In extreme cases, surgery may be required for certain scleroderma patients. For example, patients with severe Raynaud’s phenomenon in their fingers or toes may have tissues that die off or develop painful sores; consequently, amputation of these tissues may be required. Also, in patients with heavy lung involvement, a lung transplant may be necessary to help the patient breathe.
10. Scleroderma support groups are here to help
The Scleroderma Foundation offers numerous local chapters and support groups, designed to help patients connect with others living with the disease. These support groups provide a forum to share feelings, concerns, information with others, and act as a place to offer peer support and encouragement. To find your local support group in the US, visit the scleroderma chapter locator.