What Is Scleroderma?
Scleroderma means hardening and thickening (sclero) of the skin (derma). Doctors define scleroderma as a rare autoimmune disease.
In scleroderma, the immune system — which responds to injuries and infections — is overactive. The body makes too much collagen in response to an injury.
Collagen is the building block of the skin, muscles, and other connective tissues. It's also found throughout the body, such as in your blood vessels, lungs, esophagus, intestines, and heart.
Some types of scleroderma mainly affect the skin. Other types can affect the digestive system, lungs, kidneys, heart, and other organs.
Is scleroderma a serious disease?
Scleroderma can be mild, moderate, or severe.
On the mild end, it may cause skin changes, but they aren't life-threatening. On the severe end, scleroderma can damage the lungs and heart, which can cause death.
What Are the Different Types of Scleroderma?
There are two main types of the disease:
Localized scleroderma
This type only affects the skin and sometimes the muscle and connective tissue under the skin.
There are two forms of localized scleroderma:
- Morphea causes patches of hard, thick skin. These patches often first appear on the hands, feet, and face. But they can spread to other parts of the body. It's the most common form of scleroderma.
- Linear scleroderma tends to start in childhood. The thick, hard skin forms in a line or band on the arms, legs, or face.
Systemic scleroderma
This type, also called systemic sclerosis, affects the skin and other parts of the body.
It has two main forms:
- Limited cutaneous scleroderma causes the skin to thicken. It also causes problems with your blood vessels and esophagus.
- Diffuse cutaneous scleroderma comes on faster than the limited form. This severe form of scleroderma often starts in the fingers and toes but quickly spreads. It affects the skin, connective tissue, and blood vessels. It can also damage the heart, lungs, and kidneys.
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What Causes Scleroderma?
No one knows exactly what causes scleroderma.
Genes play a role, as it can run in families, but there's no one genetic test for scleroderma. And many people with scleroderma don't have a family history of the disease.
Research shows that people exposed to the chemical silica, used in manufacturing and fertilizer, are more likely to get scleroderma. But most people with scleroderma haven't had known exposures to silica or other toxic chemicals.
What Are the Risk Factors and Complications of Scleroderma?
Risk factors
Scleroderma risk factors include:
- Age, with most cases diagnosed between 30 and 50.
- Gender, as scleroderma is four times more common in women than in men.
- Race, with Black and Native Americans more likely to get scleroderma. They also tend to have more severe forms of the disease.
- Family history. Having a parent or sibling with scleroderma will increase the chances of getting it.
Complications of scleroderma
Systemic scleroderma can cause severe health problems.
Doctors will watch you closely to prevent and treat issues like:
- Ulcers or wounds in the toes and fingers due to reduced blood supply.
- Pulmonary hypertension, where the blood pressure from the heart to the lungs is too high.
- Heart failure, which means the heart can't pump enough blood for the body to function like it should.
- Inflammation in or around the heart.
- Gastroparesis, which causes stomach muscles to contract slower and affects digestion.
- Scarring of the lungs that makes it harder to breathe.
- Decreased sexual function.
- Kidney damage due to reduced blood supply to the kidneys.
- An irregular heartbeat.
- Reduced motion in the hands, feet, wrists, ankles, knees, and elbows due to hardened skin in the joints.
- Sjogren's syndrome, or a very dry mouth and eyes.
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What Are the Signs and Symptoms of Scleroderma?
Localized and systemic scleroderma symptoms can include:
- Discolored patches on the skin.
- Waxy, hardened patches of skin, especially on your hands, feet, and face.
- Strips or bands of thick, hard skin on your arms, legs, or face.
- Joint pain.
- Joint stiffness, including trouble straightening your fingers.
- Loss of hair and ability to sweat on affected skin areas.
- Muscle weakness.
On top of these symptoms, systemic scleroderma can cause:
- Skin sores and red spots on the skin.
- Heartburn.
- Indigestion.
- Feeling like food gets "stuck" in your throat when you swallow.
- Raynaud's syndrome, or blood vessels that narrow too much in response to cold or stress. This causes your fingers and toes to go numb.
- Stomach bloating, diarrhea, or constipation.
- Shortness of breath.
- Abnormal heartbeat.
- Sexual problems, like erectile dysfunction or pain during sex.
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How Do You Diagnose Scleroderma?
To diagnose scleroderma, doctors will:
- Do a physical exam.
- Look at your skin.
- Listen to your heart and lungs.
- Check your blood pressure.
- Ask about your symptoms and how they have changed over time.
Testing for scleroderma
There are tests that help with the diagnosis. Because the effects of scleroderma vary widely, tests also show doctors if it's affecting your organs.
Tests include:
- Blood tests to look for antibodies found in people with scleroderma and other autoimmune diseases.
- Esophagram, a special x-ray to study the function of the esophagus.
- Endoscopy, or a special scope with a camera on it to see if scleroderma is affecting the digestive tract.
- Pulmonary function (breathing) tests.
- High-resolution CT scan of the lungs.
- EKG to see if scleroderma affects the heart's rhythm.
- Echocardiogram to measure blood supply to and from the heart.
- Complete blood tests (CBC) and urinalysis to check kidney function.
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What Are the Treatment Options for Scleroderma?
Systemic scleroderma can affect many parts of the body.
You should get routine checkups with doctors who focus on this disease. This way, doctors can catch problems in the early stages and treat them before they cause long-term damage.
UPMC tailors its treatments to your unique needs.
Medicines to treat scleroderma
Based on your symptoms and test results, doctors may prescribe drugs:
- That suppress the immune system to treat moderate to severe forms of systemic sclerosis.
- That dilate blood vessels to treat Raynaud's syndrome.
- To reduce joint pain and inflammation.
- That block stomach acid production, for heartburn.
- That work in the digestive tract to reduce bloating, pain, diarrhea, or constipation.
- To treat high blood pressure and improve blood flow to the heart and lungs.
Exercise, physical therapy (PT), and lifestyle changes
To help people with scleroderma stay healthy and active, doctors advise:
- Exercise and PT to help maintain joint motion.
- Quitting smoking because it makes symptoms worse.
- Taking measures to protect yourself from the cold, especially your hands, feet, and head, to prevent Raynaud's syndrome.
- Eating smaller amounts at a time, if you get digestive symptoms or heartburn.
- Getting routine vaccines to prevent infections, which are more likely when taking immune-suppressing drugs. This also helps keeps the lungs healthy.
How Long Can a Person Live With Scleroderma?
Most people with scleroderma have a normal life span.
But people with severe forms of the disease can suffer fatal issues if they don't get routine tests and treatment.
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