UPMC Scleroderma Center (Systemic and Localized Sclerosis)

What Is Scleroderma?

Scleroderma literally means hardening and thickening (sclero) of the skin (derma).

There are two forms of the disease:

• Systemic scleroderma (systemic sclerosis) is capable of causing serious, potentially fatal internal organ involvement.
• Localized scleroderma affects the skin only.

Both systemic and localized scleroderma are disorders that activate the immune system, leading to tissue damage and eventually resulting in excessive deposits of collagen (fibrosis).

Doctors have identified distinct subsets of scleroderma patients, which helps them direct treatment approaches and assess future risks.

Why Choose the UPMC Scleroderma Center?

The UPMC Scleroderma Center has specialized in the diagnosis and treatment of scleroderma for more than 50 years and has evaluated and treated more than 5,000 patients. Our success at managing and treating scleroderma stems from the comprehensive care that is tailored to the needs of each patient.

Our physicians are recognized as leaders in the field, having published more than 500 articles on scleroderma in medical literature.

Scleroderma Symptoms and Diagnosis

The UPMC Scleroderma Center offers a comprehensive approach to diagnosing scleroderma. Our physicians perform an array of tests and check for symptoms related to scleroderma to ensure a correct diagnosis.

Signs and symptoms of systemic scleroderma

Common scleroderma symptoms include:

• joint pain and stiffness — particularly in the small joints of the hands — with prominent morning “stiffness” lasting over one hour
• swollen fingers and inability to straighten the fingers (contractures)
• heartburn or indigestion, as well as bread or meat getting “stuck” in the mid-chest (behind the breastbone) during swallowing
• abdominal bloating or distention within one hour after eating
• frequent watery diarrhea accompanied by weight loss
• shortness of breath on exertion
• sudden onset of severe high blood pressure with headache or blurry vision

Associated conditions

Although systemic scleroderma can affect many organ systems, 99 percent of patients have at least one of the following:

• color changes at the tips of the fingers on cold exposure or with emotional stress (Raynaud's phenomenon)
• skin thickening of the fingers (sclerodactyly)
• positive antinuclear antibody (ANA) test

A person with none of these findings is highly unlikely to have systemic scleroderma. 

Testing for scleroderma

In addition to a physical exam, the doctor may order the following tests to help determine if a patient has scleroderma:

• antinuclear antibody (ANA) blood tests — for ANA and a number of scleroderma-associated antibodies
• esophagram — x-ray to study the function of the esophagus while swallowing barium
• pulmonary function (breathing) tests
• high-resolution CT scan of the lungs
• routine electrocardiogram (EKG) and echocardiogram
• routine blood tests (CBC) and urinalysis — to check kidney function

Scleroderma Treatments

The UPMC Scleroderma Center offers a comprehensive treatment approach, tailored to the individual needs and disease subtype of each patient.

Medicines	Drugs that suppress the immune system — when internal organs are involved, or an increased risk of such involvement (systemic sclerosis). Blood vessel dilators — for Raynaud phenomenon. Anti-inflammatory drugs — for joint pain. Drugs that block stomach acid production — for heartburn.
Exercise	We often recommend a vigorous exercise program to prevent loss of joint motion and to strengthen the muscles surrounding affected joints.