Systemic sclerosis (SS) is an autoimmune disorder that causes atypical growth of connective tissues. Connective tissue gives your tissues (organs and muscles) strength and shape. With SS, your tissue becomes thick and stiff, leading to swelling and pain.

It also causes changes to the texture and appearance of your skin due to increased collagen production. Collagen is a component of connective tissue and hardens with this disease.

SS causes an immune response that destroys healthy tissue as an autoimmune disease because your body’s immune system mistakenly thinks your tissue is a foreign substance or infection .

You then see fibrosis or scar tissue, also described as connective tissue growth, in response to a perceived injury or damage.

Additionally, SS changes the texture and appearance of skin due to increased collagen production.

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

Features of SS can appear in other autoimmune disorders. When this occurs, it’s called a mixed connective tissue disease.

Scleroderma commonly affects people 30 to 50 years of age but is found in all ages. Also, women are typically more likely than men to receive a diagnosis of this condition.

Three types of SS exist, all classified by the tissues affected:

  • Limited cutaneous systemic scleroderma. The fibrosis affects only your hands, arms, and face.
  • Diffuse cutaneous systemic scleroderma. The fibrosis affects large areas of your skin, including your torso, upper arms, and legs, as well as internal organs.
  • Systemic sclerosis sine scleroderma. This is when the fibrosis affects one or more internal organs, but not your skin.

Limited cutaneous systemic scleroderma was once known as CREST syndrome. The word “CREST” stands for:

The condition worsens quickly in diffuse cutaneous systemic scleroderma, with organ damage occurring earlier than the other two types of SS.

As previously mentioned, 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.

Experts may not know a cause, but the immune system and a combination of other risk factors play a role.

Genetics

You may have a specific gene makeup making you more prone to developing scleroderma. Evidence suggests a higher incidence of SS in parents, siblings, and children.

The highest prevalence of SS in the United States is among a Native American tribe known as the Oklahoma Choctaw Indians, where they experience 469 cases per 100,000 people. This older statistic could be further evidence that genetics may play a significant factor in SS.

Environmental triggers

Exposure to certain agents may trigger SS, including:

  • viruses
  • medications
  • drugs
  • chemicals

Immune system issues

Since scleroderma is an autoimmune condition, it may occur because your immune system destroys your connective tissues.

An estimated 15 to 25 percent of people with SS also show signs of other connective tissue disorders, such as:

This overlap is also known as scleroderma overlap syndrome.

The symptoms and severity of the disorder vary from one person to another based on the systems and organs involved.

Skin

It is very common to experience hardening and tightening of patches of your skin with SS. The patches may be oval-shaped or a straight line that can cover large areas of your skin.

Your skin may also appear shiny since it’s so tight with your movement restricted.

Fingers and toes

Raynaud’s phenomenon results from limited cutaneous systemic scleroderma. Here, you will see small blood vessels in your fingers and toes start to constrict, or shrink, in response to cold temperatures or certain emotions.

As a result, your fingers and toes may become painful, turn blue, or go numb.

Digestive system

SS affects all or some parts of your digestive tract. If the esophagus is involved, you may experience heartburn or swallowing difficulty.

Your intestines may also be affected. In that case, you may experience:

In your intestines, your body absorbs the nutrients it needs to function. So if your muscles are not working correctly to digest your food, you may experience nutritional deficiencies as a result.

Heart, lungs, and kidneys

SS may affect other organs, including your heart, lungs, and kidneys. When the disease process reaches this point, it may become life threatening without early treatment.

Scleroderma renal crisis is a rare but severe complication of SS that remains a significant risk factor for the life threatening progression of the disease. However, a 2016 study mentioned that it only affects 2.4 to 5 percent of people who receive a diagnosis of SS.

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Scleroderma is an autoimmune condition that attacks the skin tissue. Numstocker/Shutterstock
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With systemic sclerosis, the skin becomes hard, thick, and tight, causing pain. Numstocker/Shutterstock

There isn’t a single test for scleroderma or SS. However, your doctor may determine your diagnosis by:

  • asking about your medical history
  • asking about your symptoms
  • asking about your family history of autoimmune diseases
  • performing a physical exam
  • ordering lab tests

By analyzing your symptoms, identifying skin changes, and reviewing lab values, the doctor may determine scleroderma or SS is the cause.

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:

Depending on your symptoms, treatment can also include:

Treatment for Raynaud’s phenomenon may include:

  • nitroglycerine 2 percent ointment as a topical treatment
  • calcium channel blockers, like Nifedipin

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

A 2019 study found the estimated 5-year survival rate for people with SS to be 85.9 percent. However, every person is different, and the outlook depends on the progression of the disease and other factors, like your current state of health, sex, and ethnicity.

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.

It may be a good idea to talk with your doctor if any of your symptoms are getting in the way of your daily life. They can work with you to adjust your treatment plan.

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

What are the first signs of systemic sclerosis?

Raynaud’s phenomenon is one of the earliest signs of SS. It causes the blood vessels in your fingers and toes to contract in response to stress or cold temperatures. As a result, your fingers and toes may turn blue or feel painful and numb.

What is the life expectancy of someone with systemic sclerosis?

After receiving a diagnosis of scleroderma, 90 percent of people have a 5-year survival rate, with 84 percent living after 10 years.

Is scleroderma fatal?

Even though there is no cure for scleroderma, most people with the condition may lead a full and happy life due to treatments available for symptom management.

What’s the difference between scleroderma and systemic sclerosis?

Scleroderma is seen only on the skin. However, when the fibrosis involves your other internal organs, this is considered systemic sclerosis.