Scleroderma is a rare rheumatic disease, in which the skin hardens or tightens and the connective tissue in the organs increases. This affects the skin, joints, blood vessels, esophagus, stomach, intestines, lungs, heart and kidneys. There are two forms of the disease. The local form only affects the skin. With the systemic form, multiple organs are involved.
As with many other autoimmune diseases, the cause of scleroderma is not known. There is vascular damage and activation of the immune system. Heredity possibly plays a role. Exposure to substances, such as vinyl chloride, silicon dust and mixed rapeseed oil, can lead to scleroderma-like diseases. Using bleomycin, an agent used in chemotherapy, can also cause a similar condition.
The local form of scleroderma is characterized by symptoms of inflammation (redness, swelling and pain) that lead to thickening or hardening of the skin. Raynaud’s phenomenon, which is characteristic of Sjögren’s syndrome, is also common. Here, the fingers or toes suddenly become very pale and start to tingle or get numb in response to cold or emotional upset. Typical are the color changes to red, white or blue that occur here.
When scleroderma progresses, the skin becomes tight, shiny and darker than usual. The facial skin gets tighter, which can lead to a mask-like face. Spider veins (telangiectasia) appear at the fingers, chest, face, lips and tongue. Furthermore, bumps made up of calcium may arise on the fingers, near the joints and other bony parts of the body.
In case of systemic scleroderma, apart from damage to the skin, acid reflux may also occur as a result of damage to the esophagus. As with Sjögren’s syndrome, scleroderma may cause difficulty swallowing. The kidneys, heart and lungs can be damaged by scarring. Thus, these organs will function worse.
The diagnosis of scleroderma is often difficult to make. This is due to the fact that in the early stages of the disease, the symptoms are often very vague. Skin problems, for example, may be absent. Therefore, the doctor makes a diagnosis not only on the basis of skin research and the symptoms, but also performs blood tests and makes X-rays. In case of scleroderma, specific antibodies may be found in the blood and be demonstrated with blood tests. X-rays are taken to see if the patient has calcium deposits at the fingertips and toes, an important indicator of the disease. Other tests that may be done are:
- Capillary microscopy. This allows the capillaries in the nail bed of the fingers to be assessed.
- CT scan. For example, in order to determine whether or not the small alveoli are inflamed.
- Cardiac ultrasound.
- Electrocardiography (ECG).
- Pulmonary function test.
- Esophageal manometry.
There is no known treatment that can cure scleroderma. Treatment is therefore aimed at reducing the symptoms and complications. Sufficient exercise is important. Exercises that don’t strain the body too much keep the joints and skin flexible. Physical therapy, occupational therapy and remedial therapy can also help to keep the body agile.
The dry skin can be treated with creams and lotions. It’s advisable to avoid aggressive soaps, detergents and too hot water. These dry out the skin further.
People with scleroderma have to wear warm or thermal clothing in cold weather.
With drugs, such as anti-inflammatories and corticosteroids, the pain and the inflammation in the joints and muscles can be relieved. With immunosuppressants, the immune system can be slowed down and inflammation in the lungs can be reduced. Raynaud’s phenomenon can be treated with drugs that dilate blood vessels and improve blood circulation.
Scleroderma does not pass. The life expectancy of people with the local form of scleroderma virtually doesn’t differ from healthy people. In case of systemic scleroderma, life expectancy is lower, depending on the extent to which the organs are affected. Seventy percent of patients with mild systemic scleroderma survives ten years. This is less when the disease is more severe or more advanced.
- Exercising is very important in rheumatic diseases, such as scleroderma. It’s healthy because it keeps muscles strong and keeps up condition.
- Exercises can help the patient to reduce limitations.