Scleroderma by Dr. Humeira Badsha

Scleroderma is a lesser known but nevertheless important rheumatic disease. The word “scleroderma” means thick skin but the disease may cause more problems than that. It is not very common and much is still not well understood about the condition. But for those who suffer from this disease it is important for you to know certain facts about this condition so that you can participate in the management of this chronic condition.
What is Scleroderma?
Scleroderma is a disease where the skin and many other parts of the body can become thick and hard. There are two kinds of Scleroderma: localised and generalised. The localised type tends to be milder and affect mainly the skin, while the systemic type may affect the internal organs.
The skin is most frequently involved. Many of the patients are sensitive to the cold and their fingers will turn white or blue when exposed to the cold (this is known as Raynaud’s phenomenon). The internal organs Iike the lung, kidney, heart, stomach and intestines may also be involved.
Signs and Symptoms
The signs and symptoms depend on the organ involved. The skin is thickened and may look shiny. Patches of the skin may become darker or lighter in colour. There may be prominent tiny blood vessels (telangiectasia) on the skin and small chalky lumps (calcinosis) under the skin. Sometimes ulcers form at the bony prominences. Cold sensitivity is often noticed by the patient when she/he goes into an air-conditioned room. Swelling of the hands and feet may be associated with aching especially in the morning. The joints may hurt and if proper exercises are not done, the hands may permanently stay crooked. Involvement of the lungs causes the patient to feel breathless especially on exercise. Involvement of the intestines may give the patient heartburn or a feeling of abdominal pains. There may also be abdominal swelling with poor appetite, vomiting and loss of weight. Involvement of the kidneys may result in sudden high blood pressure with headache and vomiting.
Blood tests may show the presence of autoantibodies like the antinuclear antibody. Autoantibodies are produced by the body’s immune system which has mounted a “rebellion” against itself.
Scleroderma affects different patients differently. Your doctor will assess you and send you for various tests to determine the extent of your disease. This is important because the treatment given will depend on the severity of the disease in individual patients. These tests include X-rays, ECG, urine and blood tests and sometimes other specialized tests. It is important to assess the heart and lungs with chest x-rays, echocardiograms, and pulmonary function tests to make sure that there are no heart and lung complications. It is also important to have regular check for blood pressure and a urine test to make sure there is no protein in the urine.
What is the Cause of Scleroderma?
The exact cause of Scleroderma is unknown. Genes are important and patients are born with the increased susceptibility to developing the disease. The presence of autoantibodies described above suggest that it is an autoimmune disease – a disease where the body is attacked by its own immune system. The blood vessels are abnormal and there is an increased production of the connective tissue resulting in the thickening of the skin and internal organs.
The treatment given will depend on the extent of the disease. Treatment includes care of the skin, treatment of skin ulcers and avoidance of the cold. The physiotherapist and occupational therapist will teach you exercises to prevent deformities and ways to protect joints. Patients should not smoke as this will aggravate the disease in the lungs.
Medicinesvmay be prescribed for Raynaud’s phenomenon and these act to open up the blood vessels but may cause a headache or worsening of heartburn. Patients with joint pains and stiffness will be given medicines which act to reduce the inflammation. Patients with heartburn should take small frequent meals and avoid big meals especially at night. It may sometimes be necessary to elevate the head of the bed as symptoms are aggravated when lying flat. Many drugs have been tested for the treatment of Scleroderma. Many of these drugs have toxic side-effects and may not be very effective. Hence your doctor will decide whether or not to recommend specific drug therapy for Scleroderma after careful evaluation. Your doctor will also discuss with you the possible side effects so that you will be aware of them.
This is one of a set of articles by Dr. Humeira Badsha, Specialist Rheumatologist, for patient awareness.

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