Scleroderma

Scleroderma is an autoimmune disease that affects your skin and the connective tissues that protect your organs and other parts of your body. It's a long-lasting autoimmune disease that causes hard skin and can lead to a buildup of fibrous tissue.

This condition can range from mild skin changes to more serious issues affecting your internal organs. Our rheumatology experts will work closely with you and your primary care doctor to help you manage this condition and help you improve your quality of life.

Systemic scleroderma

Also known as systemic sclerosis, systemic scleroderma is more widespread and can affect your skin, blood vessels and internal organs such as your heart, lungs and kidneys.

Systemic sclerosis has two subtypes that have distinct characteristics:

• Limited cutaneous systemic sclerosis: This subtype impacts your skin on the face, hands and feet. It can also cause serious issues like pulmonary hypertension, a condition that causes your heart to struggle with pumping blood into your lungs.
• Diffuse cutaneous systemic sclerosis: This subtype is more severe and can affect larger areas of your skin and internal organs. It can lead to significant tissue injury and complications such as kidney failure and heart problems.

Scleroderma symptoms

Symptoms of scleroderma may be different from one person to the next. This is mainly due to the type of scleroderma and how severe it is. The most noticeable scleroderma symptom is hard, thickened skin that may feel tight and appear shiny. Other common symptoms include:

• Raynaud's syndrome: This condition causes your fingers and toes to turn white or blue in response to cold due to reduced blood flow.
• Joint pain and stiffness: Scleroderma can cause your joints to become stiff and painful, making movement difficult. However, this can be a common symptom of other autoimmune diseases such as rheumatoid arthritis (RA), multiple sclerosis (MS) or lupus.
• Digestive problems: These can include acid reflux, difficulty swallowing and trouble absorbing vitamins and minerals. Digestive problems are also a common sign of autoimmune disease.
• Shortness of breath: If scleroderma affects your lungs, you may have trouble breathing and have other respiratory problems.
• Changes in your face: The skin on your face may become tight, making it difficult to open your mouth or smile.

What are the early stages of scleroderma?

It’s important to recognize any signs of the early stages of scleroderma so you can get a proper diagnosis and the appropriate treatment. In the early stages, scleroderma symptoms may be subtle and easy to overlook, but they often include:

• Raynaud's syndrome: This is often the first symptom people notice. It can occur years before other signs appear.
• Swelling of fingers and hands: Your fingers and hands may feel puffy and swollen, especially in the morning.
• Skin changes: You may notice patches of thickened skin, which could be a sign of localized scleroderma.
• Fatigue and general discomfort: Feeling unusually tired or experiencing unexplained aches and pains can also be early indicators of scleroderma.

If you notice any of these signs, schedule an appointment with your primary doctor. They can rule out other conditions and refer you to a rheumatologist if needed.

Complications of scleroderma

Scleroderma can lead to several complications, particularly when it affects your organs. Some of the potential complications include:

• Pulmonary complications: Systemic sclerosis can cause scarring of lung tissue, leading to pulmonary fibrosis and other breathing difficulties.
• Kidney problems: Scleroderma can affect your kidneys, leading to high blood pressure and, in severe cases, kidney failure.
• Heart problems: Scleroderma can cause scarring of heart tissue, leading to irregular heartbeats, heart failure and other cardiovascular problems.
• Stomach issues: Damage to the esophagus and other parts of the digestive system can cause significant discomfort and vitamin deficiencies.
• Scleroderma renal crisis: This rare but life-threatening complication involves a sudden development of high blood pressure and kidney failure.

Scleroderma diagnosis

Diagnosing scleroderma involves a combination of physical exams, lab tests and imaging tests. Your doctor will review your medical history, discuss your scleroderma symptoms and look for any changes in your skin, such as hard skin and Raynaud’s syndrome.

If your rheumatologist suspects scleroderma, they may order blood tests to check for specific antibodies associated with scleroderma. They may also do a skin biopsy where they take a small sample of your skin to look for excess amounts of collagen or tissue damage.

X-rays, CT scans or MRIs may be used to assess the extent of organ involvement and rule out other conditions.

Scleroderma treatment

There’s no cure for scleroderma, but several treatments can help manage the symptoms and slow the progression of the disease. Your rheumatologist will develop a scleroderma treatment plan that fits your specific needs based on the type of scleroderma and how severe it is.

Common scleroderma treatment options

• Medications: Drugs that suppress the immune system, such as methotrexate or mycophenolate, can help reduce inflammation and slow the disease’s progression. Other medications may be used to manage specific symptoms. These medications may include calcium-channel blockers for Raynaud’s syndrome or proton-pump inhibitors for acid reflux.
• Physical therapy: Regular physical therapy can help maintain joint flexibility and reduce stiffness.
• Skin care: Keeping your skin moisturized and protected from the cold can help manage skin symptoms.
• Surgical options: In severe cases, surgery may be necessary to manage complications like severe Raynaud’s syndrome or blockages in your digestive tract.

Can scleroderma go away?

Scleroderma is a chronic, long-lasting condition that doesn’t go away. Living with scleroderma can be challenging, but understanding the disease and working closely with your health care team can help you navigate its complexities.

The disease’s progression varies from person to person. Some people may experience only mild skin symptoms, while others may have more severe complications. Early diagnosis and a comprehensive treatment plan are key to managing scleroderma effectively.