Systemic Sclerosis (Scleroderma)

Written by Jaime Herndon | Published on June 29, 2012
Medically Reviewed by Brenda B. Spriggs, MD, MPH, FACP

What is Systemic Sclerosis?

Systemic sclerosis (SS) is an autoimmune disorder. This means that it’s a condition in which the body’s immune system attacks its own body. Healthy tissue is destroyed because the immune system mistakenly thinks it is a foreign substance or infection. There are various kinds of autoimmune disorders that can affect different systems of the body.

SS is characterized by changes in the texture and appearance of the skin due to an increase production of collagen, a component of connective tissue. However, the disorder is not confined to skin changes. It can affect blood vessels, muscles, and a number of vital organs such as the heart, digestive system, lungs, and kidneys. Features of systemic sclerosis can be seen in other autoimmune disorders and when this occurs, it is called a mixed connective disorder.

The disease is typically seen in individuals ages 30 to 50 years old, but can be diagnosed at any age. Women are four times more likely than men to be diagnosed with this condition. The symptoms and severity of the condition can vary among individuals and are dependent on the systems or organs involved.

Systemic sclerosis is also called scleroderma and progressive systemic sclerosis, or CREST syndrome. CREST syndrome is actually a limited form of the disorder.

Symptoms of Systemic Sclerosis

SS may affect just the skin in the early stages of the disease with thickening of the skin with tight, shiny areas around the mouth, nose, and fingers and over other bony areas.

As the skin involvement progresses, patients experience limited movement of these affected areas. Other symptoms include:

  • hair loss
  • white lumps under the skin (calcium deposits most prevalent in CREST syndrome)
  • small dilated blood vessels under the skin surface
  • joint pain
  • shortness of breath
  • dry cough
  • diarrhea
  • constipation
  • difficulty swallowing
  • esophageal reflux
  • abdominal bloating after meals

A characteristic feature of the skin changes of SS involves the blood vessels. Individuals experience spasms of the blood vessels of the fingers and toes. The extremities then turn white and blue on cold exposure or with extreme emotional stress. This is called Raynaud’s phenomenon.

Risk Factors for Systemic Sclerosis

The exact cause of SS is not known, but there are risk factors that can increase the chances of developing the condition. These risk factors include:

  • being Native American or African American
  • being female (four times greater risk than men)
  • use of certain chemotherapy drugs such as Bleomycin
  • being exposed to silica dust and organic solvents

There is no known way to prevent SS, other than to reduce the controllable risk factors (such as chemical exposure).

Diagnosis of Systemic Sclerosis

A physical exam can show skin changes consistent with SS. High blood pressure may be caused by kidney changes from sclerosis. Blood tests like antibody testing, rheumatoid factor, and sedimentation rate may be done. Other diagnostic tests can include:

  • blood tests (antinuclear antibodies, rheumatoid factor, sedimentation rate)
  • chest X-ray
  • urinalysis
  • computed tomography (CT) scan of the lungs
  • skin biopsies

Other tests may be done based on individual symptoms.

Treatment for Systemic Sclerosis

There is no one standard treatment for SS. Treatment is typically determined based on a person’s symptoms and the need to reduce the risk of complications. Treatment for generalized symptoms may involve:

  • corticosteroids
  • immunosuppressants (methotrexate or cytoxan)
  • nonsteroidal anti-inflammatory drugs

Depending on the symptoms, treatment can also include:

  • blood pressure medication
  • medications to aid breathing
  • physical therapy
  • light therapy (phototherapy UV1) and nitroglycerin ointment for localized areas of tightening of the skin

There are lifestyle changes you can make to help keep you healthy with scleroderma, including:

  • avoiding smoking cigarettes
  • remaining physically active
  • avoiding foods that trigger heartburn

Potential Complications of Systemic Sclerosis

Some individuals with SS experience progression of their symptoms. Complications can include heart failure, cancer, kidney failure, and high blood pressure. According to the National Institutes of Health, the most common cause of death in patients with systemic sclerosis is pulmonary fibrosis or scarring of the lungs (NIH, 2012).

Was this article helpful? Yes No

Thank you.

Your message has been sent.

We're sorry, an error occurred.

We are unable to collect your feedback at this time. However, your feedback is important to us. Please try again later.

Article Sources:

  • Diot, E., Lesire, V. Guilmot, JL, Pilore, R., Rogier, S., et al.Systemic sclerosis and occupational risk factors: a case–control study (February 13, 2002). Occup Environ Med 2002;59:545-549 doi:10.1136/oem.59.8.545. Retrieved September 1, 2012, from http://oem.bmj.com/content/59/8/545.full

More on Healthline

Numbness, Muscle Pain and Other RA Symptoms
Numbness, Muscle Pain and Other RA Symptoms
The symptoms of RA are more than just joint pain and stiffness. Common symptoms include loss of feeling, muscle pain, and more. Learn more in this slideshow.
Timeline of an Anaphylactic Reaction
Timeline of an Anaphylactic Reaction
From first exposure to life-threatening complications, learn how quickly an allergy attack can escalate and why it can become life threatening.
Seasonal Allergies and COPD: Tips to Avoid Complications
Seasonal Allergies and COPD: Tips to Avoid Complications
For COPD patients, allergies pose the risk of serious complications. Learn some basic tips for avoiding allergy-related complications of COPD in this slideshow.
The Best Multiple Sclerosis iPhone and Android Apps of the Year
The Best Multiple Sclerosis iPhone and Android Apps of the Year
These best multiple sclerosis apps provide helpful information and tools to keep track of your symptoms, including medication reminders.
Lifestyle Changes to Help Manage COPD
Lifestyle Changes to Help Manage COPD
Leading a healthy lifestyle can make a big difference in your COPD symptoms. Learn more about basic changes that will make it easier to manage your COPD.
Advertisement
Advertisement
Advertisement