Systemic sclerosis (scleroderma) causes the body to destroy healthy tissue. It can change the appearance and texture of your skin and affect other organs.

Systemic sclerosis (SS) is an autoimmune disorder that causes atypical growth of connective tissues. Connective tissue gives your 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 is an autoimmune disease. It destroys healthy tissue 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 old but is found in all ages. Also, females are typically more likely than males to receive a diagnosis.

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 acronym “CREST” stands for:

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

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

Your genetics may play a role in your level of risk for 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 660 cases per 1 million people.

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% 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. A 2020 study mentioned that the number has decreased in recent years and currently affects about 2% to 5% of people who receive a diagnosis of SS.

There isn’t a single test for scleroderma or SS. However, a doctor may make a 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, your doctor may determine scleroderma or SS is the cause.

Some people with SS experience a progression of their symptoms. As a result, complications can occur, which include the following:

Treatment can’t cure the condition, but it can help reduce symptoms and slow disease progression. Treatment is typically based on your 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% ointment as a topical treatment
  • calcium channel blockers, like Nifedipine

Lifestyle changes, such as avoiding smoking cigarettes, staying physically active, and avoiding foods that trigger heartburn can have a positive impact on your health as well.

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

Treatments for SS have drastically improved in the past 30 years. Although there’s still no cure, there are many different treatments that can help you manage your symptoms.

It may be a good idea to speak 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. Speaking with others who have similar experiences can make it easier to cope with a chronic condition.

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.

According to a 2019 study, the 5-year survival rate for people with SS is 85.9%. At 10 years, 71.7% were living. However, every case is different and depends on factors like your health, sex, and ethnicity.

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

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

Systemic sclerosis is a potentially serious autoimmune disorder that causes atypical growth of connective tissues. Treatment and disease management is important to avoid more serious complications. But with proper care, most people lead a full life.