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Systemic Sclerosis: Symptoms, Causes, Diagnosis, Risks, Treatment

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What is Systemic Sclerosis?

Systemic sclerosis is a rare chronic disease of unknown cause characterized by diffuse fibrosis and vascular abnormalities in the skin, joints, and internal organs (especially the esophagus, lower GI tract, lungs, heart, and kidneys). … Systemic sclerosis is about 4 times more common among women than men.
Healthy tissue is destroyed because the immune system mistakenly thinks it’s a foreign substance or infection. There are many kinds of autoimmune disorders that can affect different body systems.

Systemic sclerosis  is characterized by changes in the texture and appearance of the skin. This is due to increased collagen production. Collagen is a component of connective tissue.

But the disorder isn’t confined to skin changes. It can affect your:

  • blood vessels
  • muscles
  • heart
  • digestive system
  • lungs
  • kidneys

Features of systemic sclerosis can appear in other autoimmune disorders. When this occurs, it’s called a mixed connective disorder.

The disease is typically seen in people 30 to 50 years old, but it can be diagnosed at any age. Women are more likely than men to be diagnosed with this condition. The symptoms and severity of the condition vary from one person to another based on the systems and organs involved.

Systemic sclerosis is also called scleroderma, progressive systemic sclerosis, or CREST syndrome. “CREST” stands for:

  • calcinosis
  • Raynaud’s phenomenon
  • esophageal dysmotility
  • sclerodactyly
  • telangiectasia

CREST syndrome is a limited form of the disorder.

Causes of Systemic Sclerosis

systemic sclerosis occurs when your body begins to overproduce collagen and it accumulates in your tissues. Collagen is the main structural protein that makes up all of your tissues.

Doctors aren’t sure what causes the body to produce too much collagen. The exact cause of SS is unknown.

Symptoms of Systemic Sclerosis

SS may only affect the skin in the early stages of the disease. You may notice your skin thickening and shiny areas developing around your mouth, nose, fingers, and other bony areas.

As the condition progresses, you may begin start to have limited movement of the affected areas. Other symptoms include:

  • hair loss
  • diarrhea
  • constipation
  • difficulty swallowing
  • esophageal reflux
  • diarrhea
  • constipation
  • difficulty swallowing
  • esophageal reflux
  • calcium deposits, or white lumps under the skin
  • small, dilated blood vessels under the skin’s surface
  • joint pain
  • shortness of breath
  • a dry cough
  • abdominal bloating after meals

You may begin to experience spasms of the blood vessels in your fingers and toes. Then, your extremities may turn white and blue when you’re in the cold or feeling extreme emotional stress. This is called Raynaud’s phenomenon.

 Diagnosis of Systemic Sclerosis

During a physical exam, your doctor can identify skin changes that are symptomatic of SS.

High blood pressure may be caused by kidney changes from sclerosis. Your doctor may order blood tests like antibody testing, rheumatoid factor, and sedimentation rate.

Other diagnostic tests can include:

  • a chest X-ray
  • a urinalysis
  • a CT scan of the lungs
  • skin biopsies

Risk Factors for Systemic Sclerosis

Risk factors that can increase your chances of developing the condition include:
  • being Native American
  • being African-American
  • being female
  • using certain chemotherapy drugs such as Bleomycin
  • being exposed to silica dust and organic solvents

There’s no known way to prevent SS other than to reduce risk factors you can control.

Treatment can’t cure the condition, but it can help reduce symptoms and slow disease progression. Treatment is typically based on a person’s symptoms and the need to prevent complications.

Treatment for generalized symptoms may involve:

  • corticosteroids
  • immunosuppressants, such as methotrexate or Cytoxan
  • nonsteroidal anti-inflammatory drugs

Depending on your symptoms, treatment can also include:

  • blood pressure medication
  • medication to aid breathing
  • physical therapy
  • light therapy, such as ultraviolet A1 phototherapy
  • nitroglycerin ointment to treat localized areas of tightening of the skin

You can make lifestyle changes to stay healthy with scleroderma, such as avoiding smoking cigarettes, remaining physically active, and avoiding foods that trigger heartburn.

Potential Complications of Systemic Sclerosis

Some people with SS experience a progression of their symptoms. Complications can include:

  • heart failure
  • cancer
  • kidney failure
  • high blood pressure
What Is the Outlook for People with Systemic Sclerosis?

Treatments for SS have drastically improved in the past 30 years. Although there’s still no cure for SS, there are many different treatments that can help you manage your symptoms. Talk to your doctor if any of your symptoms is getting in the way of your daily life. They can work with you to adjust your treatment plan.

You should also ask your doctor to help you find local support groups for SS. Talking to other people who have similar experiences as you can make it easier to cope with a chronic condition.

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