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Scleroderma

What is scleroderma?  

The name scleroderma is derived from the Greek word “skleros”, which means hard, and “derma”, which means skin. The most characteristic feature of scleroderma is the build-up of tough scar-like fibrous tissue in the skin. The disease may affect the skin alone (localized scleroderma) or be throughout the body, affecting internal organs as well (systemic sclerosis).  

Localized scleroderma can be further divided into morphea and linear forms.  

Systemic sclerosis can be further divided into limited and diffuse forms. It is the degree of skin involvement that separates limited from diffuse scleroderma. People with limited scleroderma have skin thickening of their limbs but skin on their trunk and above the elbows and knees is not affected. Those with diffuse disease also have skin involvement of their upper arms and legs and their trunk. 

The course of scleroderma varies widely from person to person. The forms of scleroderma that involve major organs, such as the heart and kidney, are more severe and can possibly lead to death. The five-year survival rate of people diagnosed with severe scleroderma is 80-85%. For people with a form of the disease that affects primarily the skin, without major organ involvement, the long-term outlook is more favourable.

What are the warning signs of scleroderma?  

The symptoms of scleroderma generally differ depending on the type of scleroderma. 

Localized scleroderma 

The course of localized scleroderma is very slow and it rarely becomes more widespread or causes severe complications. Localized scleroderma takes on two forms: morphea and linear. 

With the morphea form of localized scleroderma, hard round or oval patches develop on the skin. These patches tend to be white with a reddish area around them. They may occur on your chest, stomach, face, arms, legs or other parts of your body. 

With the linear form of localized scleroderma, a line of thickened skin may occur in areas such as your face (often the forehead), arms or legs. This line of hard skin extends deep down into the skin, bones and muscles, and may alter growth of the affected part. This form usually occurs in young people who develop scleroderma. 

Systemic sclerosis 

Systemic scleroderma takes on two forms: limited scleroderma and diffuse scleroderma. 

With limited scleroderma, your skin hardening may be widespread, but not everywhere on your body, and it will not appear on your trunk. Often with this form the heart and kidneys are not involved, but heartburn from involvement of the esophagus (the swallowing tube) is a common symptom. Limited scleroderma used to be known as CREST syndrome, but most doctors don't use this term anymore. A range of conditions (and therefore symptoms) can be associated with limited scleroderma.  

  • Calcinosis is the name given to the condition where small white chalky lumps made up of calcium form under the skin. These lumps often form around the fingers but can occur anywhere in the body. They can rupture through the skin and cause a chalky white material to ooze out.  
  • Raynaud's phenomenon is caused by a clamping down of the blood vessels, especially in the hands and feet. Exposure to the cold or emotional stress can trigger such spasms. The resulting reduction in blood flow causes your fingers and toes to become white then blue then red. If the condition is severe you may also have pain and numbness in the affected areas.
  • Scleroderma can affect the esophagus (the swallowing tube). Food is carried from your mouth down your esophagus to your stomach. Muscles in your esophagus help to move the food along. With scleroderma your esophagus can become less mobile and the muscle action can diminish. Over time, the muscle may be replaced by scar tissue. This can result in difficulty swallowing, development of heartburn, a feeling of bloating when eating and a desire to vomit after eating. 
  • Scleroderma can cause hardness of the digits (sclerodactyly). With this condition the skin of your fingers may become dry, coarse-textured or feel 'woody.' The hair and the creases may disappear and fingers may look shiny. Your fingers may also stiffen and tighten into a bent position. 
  • Telangiectasia is a condition where small red spots appear on the surface of the skin. These are due to enlarged (damaged) blood vessels. They commonly occur on the fingers, palms, face, lips and tongue. 
  • Scleroderma can also cause bowel dysfunction, which leads to an inability to absorb nutrients from food, an overgrowth of bacteria in your bowel, weight loss and diarrhea or constipation. Bowel dysfunction occurs when the muscles of your intestine weaken and are replaced by scar tissue. 
  • High blood pressure on the right side of the heart can occur (called pulmonary arterial hypertension). This can be difficult to detect early so you're your doctor will have annual ultrasounds. 

With diffuse scleroderma, you may have widespread thickening of the skin on your arms, legs, face, chest and stomach. These skin changes can occur quite rapidly, though progress of this form of the disease varies widely from person to person. In addition lungs, kidneys, heart, blood vessels, bowel, esophagus, joints, and connective tissues such as tendons and ligaments are frequently involved. Diffuse scleroderma can become very severe, even life threatening. It may overlap with other autoimmune diseases, including lupus and polymyositis. If this occurs the disorder is then referred to as mixed connective tissue disease. 

Heart and lung problems may result from diffuse scleroderma. Problems with your heart could include a slowing of your heart rate or changes in its rhythmic pattern. If your lungs are involved, they will lose their ability to function efficiently. Symptoms of heart or lung involvement may include shortness of breath, coughing, chest pain and irregular beating of the heart. 

If your kidneys are affected, blood flow to them may be blocked. This can produce high blood pressure and kidney failure and is a serious problem requiring urgent treatment. Symptoms could include severe headache, shortness of breath, vision problems, chest pain and mental confusion. 

The glands and organs that produce fluid can also be affected. Dry eyes, mouth or vagina may indicate that you have a condition called Sjögren syndrome, which may accompany scleroderma. Your eyes may become dry if the glands that produce tears fail. The salivary glands in your mouth can also fail. In a woman with diffuse scleroderma, the cells that normally keep your vagina moist may fail and the area can become abnormally dry.  

The joints are not usually the primary area of involvement in scleroderma, but inflammation of the joints can occur as part of the disease. The skin, when involved with thickening and tightness, also lead to stiffness and reduced motion of the joints in the affected areas.

How is scleroderma diagnosed

Diagnosis may be difficult as there is no specific test that confirms scleroderma is present. Your doctor will probably perform a physical examination and order x-rays and blood tests. 

If significant skin changes are already present the diagnosis may be clear. Where scleroderma is suspected but skin changes are not as obvious, your doctor may wish to do a skin biopsy, which involves removing a small piece of skin to be analyzed. Your doctor may check your skin for thickened and hardened areas, and may also press your tendons and joints to detect crackling or grating sensations, which can indicate changes beneath the skin. A major clue to diagnosing scleroderma is the symmetrical (happening in the same spot on both sides of the body) hardening and thickening of the skin in areas on the fingers and toes. By looking at your fingernails underneath a microscope, your doctor may also be able to detect changes in your blood vessels that are characteristic of scleroderma and mixed connective tissue disease. 

There are a wide variety of other tests possible, which may be done based on the organs that are involved.

What are the risk factors for scleroderma? 

People who develop scleroderma are born with the genetic potential to develop the disease. Something then triggers the immune system, which normally protects the body from germs, viruses and bacteria, to attack healthy tissue in different parts of the body. This response produces inflammation and an overproduction of tough fibrous material that helps construct connective tissues, such as tendons, bones, and ligaments as well as scar tissue. This fibrous material is deposited in the skin and internal organs. What triggers this process is unknown. Scleroderma may be associated with exposure to, and high build-up in the body of, certain chemicals or heavy metals.

How common is scleroderma? 

Scleroderma is a relatively rare disease. It affects women three to five times more frequently than men. The chance of developing scleroderma increases with age, but symptoms usually begin between the ages of 30 and 50 years. In children, localized scleroderma (rather than systemic sclerosis) is more common.

Medication 

At this time there is no cure for scleroderma. Therefore treatment is designed to control the symptoms. Establishing the correct diagnosis is important most therapies work best when started early in the disease. 
If you are diagnosed as having scleroderma, the goal of your treatment plan will be to bring the symptoms and disease under control. Treatment plans are based on the type and severity of symptoms, and are individualized to meet each person's needs. Your active involvement in developing your prescribed treatment plan is essential. 

The optimal treatment is what is best in each individual person and these medications can be very complex, so speak with your doctor and/or pharmacist about what kind of medications are most appropriate for you. 

To explore this area of treatment, The Arthritis Society has developed a comprehensive expert guide that delivers detailed information on medications. Many medications used to treat arthritis are also used to treat scleroderma. 

EXPLORE: Arthritis Medications – A Reference Guide

Surgery

Where lack of blood circulation is a marked problem with scleroderma, surgical sympathectomy is sometimes done. During this procedure the nerves in the spine that causes spasms in the blood vessels are divided to lessen the chance of future spasms. 

For individuals with advanced lung or kidney disease, lung transplant or kidney transplant may be options.

Physiotherapy  

A physiotherapist (PT) can develop an individualized program that is designed to help you increase your strength, flexibility, range-of-motion and general mobility and exercise tolerance through a wide variety of therapeutic treatments and strategies. These include exercise prescription, physical interventions, and relaxation, in addition to advising you on other techniques for reducing pain and increasing your overall quality of life. PTs can also refer you to other health professionals and community services for further measures that will help you adapt to your changing circumstances.

Occupational therapy  

An occupational therapist (OT) can analyze everything you do in a day and develop a program to help you protect your joints and minimize fatigue. These healthcare professions have advanced training from a university and are registered to practice by their provincial/ territorial association. If necessary, they can help you redesign your home or workplace to make it easier for you to work or simply get around. They can also make or recommend a number of different splints, braces, orthopedic shoes and other aids that can help reduce your pain and increase your mobility and functionality. Their goal is to prepare you, using assistive devices and adaptive strategies, to reclaim as much of your former life as possible. 

Body and skin care

If you have scleroderma you should do things to care for your skin. Using a humidifier in your house during winter months will help prevent the air, and your skin from becoming too dry. Topical creams can be used to prevent your skin from drying out. Light mineral oil, petroleum jelly, and cocoa butter are moisturizers that work well. It may also be necessary for you to limit the frequency of bathing to preserve your natural skin oils. 

Try to protect areas of skin calcification from pressure and abrasion. In these hardened areas the skin can more easily break down and get infected. Keep areas of skin that may have opened very clean. Aggressive treatment of infections, should they occur, is important. 

Another goal of skin protection is to maintain good circulation. By wearing gloves and warm socks in cold weather, your skin is protected from cold air, which reduces the risk of the blood vessels shrinking (vasospasm). If reaching into the freezer for food triggers Raynaud's phenomenon try wearing gloves or oven mitts during this activity. If you have the symptoms of Raynaud's phenomenon and smoke, stop smoking. Smoking triggers vasospasms in your whole body and will worsen the symptoms. 

By carefully and adequately chewing your food you can minimize your difficulty swallowing. Drinking fluids with food will assist if the mouth is dry. There are some medications that can assist in stimulating the muscle action of the esophagus. If your esophagus has narrowed, it is possible to have it periodically stretched. 

Raising the head of your bed, and not lying down right after meals can help you minimize acid coming back up into your esophagus. Eating smaller meals more frequently will also help. 

If you have the symptoms of Sjögren syndrome, there are a variety of lubricants you can use to make up for the lack of secretions. For example, artificial tears can be used as drops in your eyes. There are also mouthwashes and sprays available to increase the moisture in your mouth. 

Where dryness of the mouth is pronounced, it is recommended that you have regular dental checks as there may be less flushing of the teeth than is usual. This can lead to increased tooth decay.

Exercise 

Regular exercise will help you to keep the blood flowing in areas that are affected by scleroderma. 

Muscles and the other tissues that hold joints together weaken when they aren't moved enough, so the joint loses its shape and function. Doing certain exercises can help keep your muscles strong and joints moving. 

There are different types of exercises:

  • Range of motion exercises reduce stiffness and help keep your joints moving. A range of motion exercise for your shoulder would be to move your arm in a large circle. 
  • Strengthening exercises maintain or increase muscle strength. 
  • Endurance exercises strengthen your heart, give you energy and keep your body flexible. These exercises include walking, swimming and cycling. 
  • Moderate stretching exercises help relieve the pain and keep the muscles and tendons around an affected joint flexible and strong. 

Always consult your doctor before beginning an exercise program. 

Protect your body and joints  

Protecting your joints means using them in ways that avoid excess stress. Benefits include less pain and greater ease in doing tasks. Three main techniques to protect your joints are: 

  • Pacing, by alternating heavy or repeated tasks with easier tasks or breaks, reduces the stress on painful joints and allows weakened muscles to rest. 
  • Positioning joints wisely helps you use them in ways that avoid extra stress. Use larger, stronger joints to carry loads. For example, use a shoulder bag instead of a hand-held one. Also, avoid keeping the same position for a long period of time. 
  • Using helpful devices, such as canes, luggage carts, grocery carts and reaching aids, can help make daily tasks easier. Small appliances such as microwaves, food processors and bread makers can be useful in the kitchen. Using grab bars and shower seats in the bathroom can help you to conserve energy and avoid falls. 

Relaxation  

Developing good relaxation and coping skills can give you a greater feeling of control over your scleroderma and a more positive outlook. 

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Contributors

This information was last updated September 2017, with expert advice from:

Sindhu R. Johnson, MD PhD
Assistant Professor, Division of Rheumatology, Department of Medicine, Faulty of Medicine, University of Toronto
Director, Toronto Scleroderma Program, University Health Network

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