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Diseases > Systemic Sclerosis | Scleroderma > Symptoms | Systemic Sclerosis | Scleroderma

Symptoms | Systemic Sclerosis | Scleroderma

There are two forms of systemic sclerosis, depending on the extent of skin involvement. They are called limited an diffuse. In the limited form, the skin on the arms from the elbows down, on the legs from the knees down, and on the face and neck may be affected. Often only the skin of the fingers and the face and neck are affected. In the diffuse form, skin involvement is more widespread and can affect the lower arms and legs as well as the trunk, back, thighs and upper arms.

When the skin tightens it takes on a shiny appearance. It can lose pigment in some areas and gain pigment in others giving it a “salt and pepper” appearance. The skin can tighten on the face causing a loss of wrinkles. Tightening of the skin on the face and around the mouth can make it difficult to properly open your mouth. The fat under the skin can disappear so the cheeks may look hollow.

Systemic sclerosis can affect other organs. In some people, but not all, the lungs can become affected. There are two main patterns of lung problems doctors look for. The first is inflammation in the lung tissue resulting in scarring of the lungs. This is called pulmonary fibrosis. The second is fibrosis and narrowing of the blood vessels running through the lungs. This causes high blood pressures in the lungs. This is called pulmonary hypertension.

Systemic sclerosis can also affect the kidneys causing kidney damage and high blood pressure. In some cases the blood pressure can rise very quickly and can be a medical emergency. It is important to follow your blood pressure closely if you have recently been diagnosed systemic sclerosis and especially if you have the diffuse type.

Systemic sclerosis can also affect the esophagus (swallowing tube), which is the tube that connects the mouth to the stomach. This can make swallowing food more difficult and can make it easier to choke on food. It can also lead to significant heartburn. Sometimes, other parts of the digestive tract such as the bowel can be affected. This can lead to trouble digesting food and malnutrition. Sometimes too many bacteria grow in the small bowel and this can cause bloating, possibly constipation and then foul smelling or severe diarrhea. If this problem is suspected, it is often treated with antibiotics.

Almost all people with systemic sclerosis have a condition called “Raynaud’s phenomenon.” This causes the fingers and toes or the tips to turn white and then blue and/or red in the cold. This happens because the blood vessels in the hands and feet over-react to cold temperatures and constrict (tighten) reducing the flow of blood. In people with limited systemic sclerosis, Raynaud’s usually starts years before skin symptoms are noticed. With diffuse systemic sclerosis, Raynaud’s usually comes on around the same time as the skin symptoms. Raynaud’s in people with systemic sclerosis is usually far more severe than people who have Raynaud’s without systemic sclerosis (3 to 5% of the population). Sometimes, painful ulcers can develop on the fingertips.

When small blood vessels are damaged, the body tries to make new ones. When this happens, you may notice tiny little red dots on the skin. These are called telangiectasia and often appear on the palms of the hands, fingers and around the mouth.

Fatigue is a common complaint in people with systemic sclerosis. You can think of it like the body’s immune system is “turned on.” When the immune system is turned on it makes you feel very tired. It’s like you’re fighting the flu.

The joints and tendons can be inflamed in people with systemic sclerosis. This is known as inflammatory arthritis. All the fingers may be swollen like sausages. It is common to have swelling at the wrist which can compress a nerve causing numbness and tingling of the fingers (carpel tunnel) at the onset of systemic sclerosis.

Some people with systemic sclerosis can also have features of other connective tissue diseases. Some examples include dryness of the eyes or mouth and muscle inflammation with muscle weakness.

Work and systemic sclerosis

Systemic sclerosis can cause stiffness, pain, fatigue and impaired function especially the hands. This can often limit people’s normal activities, including work. But there are things you can do to lessen the impact of systemic sclerosis on your work and daily routine. People with systemic sclerosis often have to learn to balance their activities with the need to rest and conserve energy. Try to keep your stress levels and daily activities balanced, and avoid too many “ups and downs.”

Adjusting features of your workplace can help make working with systemic sclerosis easier. For example, adjusting the position of chairs and desks for proper posture can help. You can also make adjustments to the seat of your vehicle to make driving more comfortable and reduce the stress on your joints. However, some people with systemic sclerosis are unable to work.

Travel and systemic sclerosis

Traveling is still possible when you have systemic sclerosis. However, it is important to discuss your travel plans with your health care provider. It is best to be organized prior to your trip to ensure a smooth, comfortable, and enjoyable time. See our travel check list.

Sex and systemic sclerosis

The discomfort, pain, fatigue, and emotional hardship with systemic sclerosis can certainly result in a loss of sex drive. Women with systemic sclerosis may sometimes have a dry vagina that can make having sex uncomfortable. Sore joints can also create difficulty with sexual intimacy.  These hardships can create barriers to sexual needs, ability and satisfaction. Take comfort knowing that sex and intimacy can be maintained in people with systemic sclerosis … it can even draw partners closer together, especially through improved communication and understanding.

For more information on intimacy and arthritis, a great book is Rheumatoid Arthritis: Plan to Win by Sheryl Koehn, Taysha Palmer and John Esdaile. Many of the tips can also be applied to people who have systemic sclerosis, whether their joints are involved or not.

Life expectancy and systemic sclerosis

Life expectancy in patients with systemic sclerosis depends on the extent and severity of internal organ involvement. Life expectancy has improved a lot due to better treatment, supportive care, and better nutrition. If you have systemic sclerosis you may benefit from a referral to a specialty centre. Specialty centres have expertise in treating the various complications of the disease.

Pregnancy and systemic sclerosis

In the past, pregnancy was discouraged in people with systemic sclerosis because they were thought to be at high-risk for complications. However, with careful planning, close monitoring, and appropriate treatment, women with systemic sclerosis can have successful pregnancies. To best avoid complications, women with systemic sclerosis should plan pregnancy when the disease is stable. Women wanting to get pregnant should discuss their plans with their health care provider and especially their scleroderma specialist.

Read more – Treatment of Systemic Sclerosis