Scleroderma is a rare disease of the connective tissue. It can cause the tissue in skin, joints, and internal organs to thicken and stiffen. While there's no cure for this autoimmune disorder, experts at UVA Health can design a treatment plan to help relieve your symptoms.
Treatment for Scleroderma at UVA Health
At UVA Health, you'll have a team of experts to design a treatment plan. You'll get care tailored based on your type of scleroderma and what part of your body it impacts, including:
Joints & Muscles
- Aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs)
- Corticosteroids
Esophagus & Digestive Tract
- Medications to limit acid production in the stomach
- Small, frequent meals
- Sleeping with your head elevated to avoid acid reflux
Skin & Internal Organs
- Corticosteroids
- Immunosuppressants
- Phototherapy
Kidney Problems &/or High Blood Pressure
- ACE inhibitors, or other antihypertensive drugs
- Antineoplastic agents
- Vasodilators
Raynaud's Phenomenon
- Calcium channel blockers
- Proper shelter and clothing to avoid cold
- Not smoking
Restricted Mobility
Physical therapy and exercise are important to maintain circulation, joint flexibility, and muscle strength.
Learn more about our expertise in autoimmune disorders.
What Are the Types of Scleroderma?
Overproduction of collagen and other connective tissue proteins are the main features of scleroderma. It's not clear what causes this overproduction. Malfunction of the immune system may contribute to excess collagen production.
There are three major forms of the disease:
- Localized scleroderma (can be morphea or linea) — Usually affects only the skin in isolated parts of the body. This form is less serious.
- Systemic scleroderma — Affects widespread areas of skin and/or internal organs, most often the lungs. Certain categories of this form of scleroderma are more serious and can be fatal.
- Overlap syndrome — May involve features of scleroderma and features of other autoimmune syndromes.
Diagnosis
The doctor will ask about your symptoms and medical history. A physical exam will be done. Diagnosis is based on changes in the skin.
Tests may include:
- Blood tests
- Esophagus motility study
- Biopsies of skin and other tissues
Imaging tests take pictures of internal body structures. These may include:
- X-ray
- MRI
- CT scan
Scleroderma Symptoms
Localized
Symptoms are usually restricted to the skin. This disorder does not progress to involve internal organs. The skin lesions may completely reverse themselves in a few months or a few years. In some cases, they lead to permanent disfigurement. Symptoms include:
- Hard patches on the skin, most often on the face or trunk (morphea)
- Lines of thickened skin that can extend to underlying muscles and bones (linear scleroderma or linear morphea)
Systemic Scleroderma
This form of the disease is typically categorized as either limited or diffuse disease. Many cases of limited disease begin gradually with Raynaud's phenomenon. This involves swelling, tingling, numbness, blue and white color, and pain in fingers and toes. It is brought on by cold or emotional distress. The condition can progress over the years to thickened skin.
Raynaud's Phenomenon Symptom
Diffuse scleroderma comes on more suddenly and can progress to involve not only the skin but the internal organs. Other symptoms of diffuse scleroderma may include:
- Diffuse thickening and hardening of the skin
- Joint and muscular pain, stiffness, and swelling
- Problems with breathing, swallowing, and digesting food due to thickening and hardening of lung, esophagus, bowel tissues
- Inflammation and thickening of large and small blood vessels
Complications of this type can affect virtually every system of the body. Common complications include:
- Bleeding
- Pulmonary hypertension
- Heart rhythm problems
- Heart failure
- Kidney failure
- High blood pressure
Who's at Risk?
Scleroderma is more common in women. The morphea type usually strikes people around 20-40 years old. Linear often occurs in children. Systemic is more likely to occur in people aged 30-50 years old.
Other factors that may increase your chance of developing this disease include:
- Family members with autoimmune disorders
- Occupational chemical exposure, such as silica, ketones, or welding fumes