Scleroderma

(Systemic Sclerosis, Morphea)

In this condition factsheet:

• The Facts
• Causes
• Symptoms and Complications
• Making the Diagnosis
• Treatment and Prevention

The Facts

Putting the two Greek words sclero (meaning hard) and derma (meaning skin) together paints a picture of the condition known as scleroderma. In this condition, the skin becomes thicker and harder.

Scleroderma belongs to the same family of autoimmune diseases as lupus and rheumatoid arthritis. It shares many features with these illnesses, including the fact that it often appears in adult women. Women are 3 to 8 times more likely to develop this condition than men are.

Another thing it has in common with these diseases is that it can cause a wide range of different symptoms and complications. For some people, it's a lifelong nuisance; for others, it's a disease that quickly worsens and may lead to death. This disease is not contagious, and it is not inherited.

About 16,000 Canadians live with scleroderma. It can affect a specific area of the body, usually the skin (called localized scleroderma). It may also affect several areas of the body, including internal organs (called systemic or generalized scleroderma).

Two forms of localized scleroderma are morphea and linear scleroderma. They usually affect only the skin, but may also affect muscles and bones.

Generalized or systemic scleroderma also has 2 forms: limited scleroderma (or CREST syndrome) and diffuse scleroderma.

Most people with scleroderma develop the condition between the ages of 30 and 50 years, although it may occur in children and seniors.

Causes

The exact cause of scleroderma is unknown. Scleroderma is a rare autoimmune disease, a disease in which the immune system attacks its own healthy cells, causing damage.

Normally, the immune system helps the body deal with infection and injury. People with scleroderma produce too much collagen, a protein that makes up connective tissue in tendons and ligaments. Depending on the type of scleroderma a person has, the extra collagen is deposited in either the skin or in other organs in the body, or both, which causes hardening of the tissues.

Symptoms and Complications

Symptoms of scleroderma include:

• thickening or hardening of the skin
• numbness and pain in the extremities brought on by cold or emotional stress
• loss of flexibility due to gradual hardening and tightening of the skin, especially on the arms, face, or hands
• joint stiffness or pain (elbows, knuckles)
• digestive problems including difficulty swallowing, problems absorbing nutrients and less muscle activity in the intestine causing constipation or diarrhea
• curling and stiffening of the fingers
• puffy hands and feet, most often noticed in the morning

There are 2 types of generalized scleroderma:

• CREST syndrome (also called limited scleroderma)
• diffuse scleroderma

The 5 main features of CREST syndrome include:

• Calcinosis – this is the formation of small, hard, white deposits in the skin, typically at the fingers, toes, knees and elbows.
• Raynaud's phenomenon – this occurs when the fingers lose their blood supply and go numb at the slightest sign of cold. Raynaud's phenomenon can exist on its own (called Raynaud's disease), or be caused by other conditions like arthritis. When it appears together with sclerodactyly (thickening and tightening of the skin of the fingers and toes), it's usually a sign of scleroderma.
• Esophageal dysfunction – people rarely have this as an early symptom, but it commonly develops a few years into the disease. The esophagus is the tube linking the mouth to the stomach. A valve at the bottom of the tube called the lower esophageal sphincter keeps acid in the stomach. When this valve does not work properly, acids from the stomach can enter the esophagus causing heartburn (gastroesophageal reflux disease) or acid reflux. The lower esophageal sphincter is the inner organ most likely to be attacked by scleroderma. Even in mild forms of scleroderma, heartburn problems are very common. Damage to the esophagus can lead to bleeding, ulcers, holes, and scarring in the esophagus. This can make it difficult to swallow, and many people with scleroderma have trouble eating. It can also cause changes in the esophageal tissue that can lead to cancer. Up to one third of people with esophageal symptoms in scleroderma develop Barrett's esophagus, a harmless condition in itself, but considered to be a precancerous growth.
• Sclerodactyly – along with Raynaud's phenomenon, this is one of the two symptoms most likely to be the first sign of scleroderma. It is a thickening of the skin of the fingers or toes.
• Telangiectasia – these are tiny red "spider veins" that typically appear on the hands and face.

Diffuse scleroderma is the other form of systemic scleroderma. In diffuse scleroderma, people experience thickening of the skin of the arms, legs, face, chest, and stomach. Inside the body, damage to the lungs, kidneys, heart, blood vessels, bowel, esophagus, and joints may occur. If many of these organs are affected, the condition may become life-threatening. This more serious form of scleroderma is occasionally fatal, and nearly always debilitating.

The most common forms of damage from diffuse scleroderma are:

• gastrointestinal – heartburn caused by hardening of the esophagus, low absorption of nutrients, and abnormalities in the way food moves in the intestine may happen to some people with scleroderma.
• joint – not so much to the bone itself as to the skin, tendons, and other tissue. This can cause the joints to lock in place when flexed. Tendinitis and a crackling sound when you move are also possible.
• lung – scleroderma can cause pulmonary fibrosis, a scarring of the lungs that interferes with the way oxygen is transferred. The scarring can lead to pulmonary hypertension (high blood pressure in the blood vessels going from the lung to the heart). This can in turn damage the heart, leading to serious, irregular heartbeats, and sometimes heart failure or sudden death.
• heart – scleroderma can also attack the heart directly, replacing healthy muscle tissue with scar tissue.
• liver – the biliary ducts that drain the liver can be scarred, possibly damaging the liver itself.
• kidney damage – kidney failure can lead to death in scleroderma when these internal organs are affected.

Many of the most severe complications of scleroderma are likely to occur in the first 5 years or not at all. People with severe forms of scleroderma have about an 80% to 90% chance of surviving 5 years after being diagnosed with the condition.

Making the Diagnosis

There's no test that will give a guaranteed diagnosis of scleroderma while excluding other diseases. The doctor must balance the symptoms, history, and examination against the often-confusing evidence from blood tests and samples of skin to come up with an idea of what condition is present. At first, the condition may look similar to other autoimmune conditions including rheumatoid arthritis or lupus.

Once scleroderma has been diagnosed, the doctor can determine whether it is localized or generalized. The presence of certain antibodies helps to identify which type of scleroderma is present.

Treatment and Prevention

There is no cure for scleroderma. However, there are medications that can reduce symptoms and organ damage.

Limited scleroderma is usually treated with topical (applied to the skin) treatments such as moisturizers or topical corticosteroid creams. Generalized scleroderma (involving the internal organs) may be treated with medications that improve blood flow, promote esophagus and bowel function, keep the kidneys functioning, and control high blood pressure.

Here are some recommendations to reduce the risk of problems caused by Raynaud's phenomenon:

• Keep warm in cold weather by dressing appropriately (e.g., wear gloves or mittens).
• Try to avoid stressful situations, as stress can trigger an attack.
• Avoid or quit smoking because the nicotine found in cigarettes can decrease skin temperature and cause blood vessels to narrow.
• Exercise to keep the body flexible and improve blood circulation. Range of motion exercises can help keep skin and joints flexible. Physical and occupational therapy may also be helpful.

Medication treatment with calcium channel blockers may be prescribed to treat Raynaud's phenomenon. They work by causing blood vessels to expand, and this improves blood flow in the skin.

Symptoms such as joint stiffness and pain can be relieved with anti-inflammatory medications such as acetylsalicylic acid* (ASA), NSAIDs (non-steroidal anti-inflammatory drugs), or corticosteroids. Certain medications called DMARDs (disease modifying antirheumatic drugs) are often prescribed along with NSAIDs. Both help reduce joint inflammation. Another class of medications called immunosuppressants may be used to suppress the immune system and prevent inflammation.

For digestive problems associated with scleroderma, antacids and antibiotics (as prescribed by a doctor) may be helpful. The classes of medications known as H2 blockers and proton pump inhibitors can help reduce damage due to gastroesophageal reflux. The esophagus can begin to narrow, and may have to be physically stretched from time to time with an inflatable tube device called a balloon catheter.

Antibiotics can prevent bacterial infection of damaged parts of the intestines.

Kidney problems used to be the major cause of death in scleroderma, but medications called ACE inhibitors can help prevent life-threatening high blood pressure caused by kidney damage related to scleroderma. In the case of complete renal (kidney) failure, a dialysis machine may be needed.

Interstitial lung disease (scarring of lung tissue) can be treated with immunosuppressant drugs. Pulmonary hypertension (high blood pressure in the lungs) can be controlled with drugs that reduce blood pressure in the lungs such as epoprostenol, bosentan, riociguat, and sildenafil.

Although there is no cure for scleroderma, there is ongoing research focusing on medications that would slow the progression of the disease and ease symptoms. 

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