Systemic sclerosis (SS) is an autoimmune disorder. This
means it’s a condition in which the immune system attacks the body. 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.
SS 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
- blood vessels
- digestive system
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:
- Raynaud’s phenomenon
- esophageal dysmotility
CREST syndrome is a limited form of the disorder.
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
- calcium deposits, or white lumps under the skin
- small, dilated blood vessels under the skin’s
- joint pain
- shortness of breath
- a dry cough
- difficulty swallowing
- esophageal reflux
- 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
Causes of Systemic Sclerosis
SS 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.
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
- being exposed to silica dust and organic
There’s no known way to prevent SS other than to reduce risk
factors you can control.
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
for Systemic Sclerosis
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:
- immunosuppressants, such as methotrexate
- 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
- 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.
Complications of Systemic Sclerosis
Some people with SS experience a progression of their
symptoms. Complications can include:
- heart failure
- 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.