Systemic Sclerosis – What is Scleroderma Disease?

Scleroderma or systemic sclerosis is a rare rheumatic disease that causes hardening of the skin and connective tissues of the person. Hardening and thickening of the skin can lead to symptoms on the face, hands, feet, and elsewhere on the body. In addition, the connective tissue in the inner parts of the body is also affected by this disease.

Connective tissue is the cornerstone of many structures such as tendons, cartilage, and blood vessels. Our organs are shaped, supported and held in place by connective tissue. The thickening and hardening of the connective tissue creates an appearance similar to scar tissue after a wound or burn. Hardening of the connective tissue with scleroderma can disrupt the normal functioning of organs such as muscles, blood vessels, joints, lungs.

While most people with scleroderma have only mild complaints, sometimes serious internal organ involvement and problems can occur. It is recommended to go to a regular rheumatology examination at least once a year for the early detection of these problems.

Scleroderma, or systemic sclerosis, is an autoimmune disease. The immune system is activated against the body’s own tissues, which it should not normally react to. The reason why it is called systemic sclerosis is that it is a disease that can affect the whole body.

Types of Systemic Sclerosis

It is a disease that is more common in women than men. It most commonly begins between the ages of 25-55, but can also occur in younger and older people. There are two main types: diffuse and limited. Diffuse systemic sclerosis involves the skin throughout the body and is more likely to affect the internal organs. Limited systemic sclerosis only affects the face, extremities of the arms and legs. The rate of progression is slow and generally milder than the diffuse type, but it can still affect internal organs such as the lungs and intestines. The condition called localized scleroderma is different from systemic sclerosis. In localized scleroderma, only the skin is affected.

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Cold Sensitivity – Raynaud’s Phenomenon

Raynaud’s (reyno) phenomenon, expressed as cold sensitivity, is one of the symptoms seen in people with scleroderma. In a cold environment, the fingers or toes first turn white and then bruise. Going outside in cold weather, being in a cold room, or even reaching for the fridge or freezer can trigger this. Stressful environments can also lead to this. The color returns to normal when the hands and feet warm up. Raynaud’s phenomenon is caused by constriction of blood vessels and reduced blood flow to the fingers.

Many people with Raynaud’s phenomenon do not have systemic sclerosis. However, the majority of people with systemic sclerosis have this symptom, and it is often the first sign of the disease. Raynaud’s phenomenon can be seen many years before the onset of the disease.

Skin Findings

  • Thickening and hardening of the skin of the hands, arms and face
  • Swelling in the hands and feet, especially in the morning
  • Loss of normal lines on the skin and brightening of the skin
  • Difficulty opening the mouth wide with the hardening of the facial skin
  • Small red dots on arms, hands and face

More rare symptoms:

  • Thinning lips
  • Thinning of the skin on the toes and soles of the feet
  • Peeling, cracking and scar formation caused by insufficient blood flow to the skin
  • White bumps due to calcium deposits under the skin, especially on the fingertips

Digestive Problems

The weakening of the esophagus muscles can make it difficult to swallow food. The frequency of reflux, which is the escape of stomach acid into the esophagus, increases. Complaints such as bloating, diarrhea or constipation may occur due to the involvement of the intestines.

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Pain and Stiffness in Joints and Muscles

Joint movements may be restricted due to hardening of the tissues around the joint. Joint swelling and pain may occur. Muscle weakness may occur.

Other Problems

One in five people with systemic sclerosis may also have another rheumatic disease, such as lupus or Sjögren’s syndrome.

In most patients, the symptoms are mild and progress slowly, after a few years the symptoms settle and the progression may stop or even improve. However, in some people it progresses quickly.

Lung involvement can cause shortness of breath and dry cough. Affecting the blood vessels in the lungs can lead to hypertension in the pulmonary circulation (pulmonary hypertension), increasing the load on the heart. Problems in the kidney can cause hypertension in the general circulation.

How Is It Diagnosed?

Persons with suspected systemic sclerosis are referred to a rheumatologist. There is no single specific test for diagnosis. Medical history and physical examination findings form the basis. Disease-specific antibodies can be checked in the blood. Tests such as chest x-ray or tomography, pulmonary function test, echocardiography, endoscopy, skin biopsy, capillaroscopy are used to diagnose and determine the organs affected by the disease.

Treatment

Although there is no treatment that completely eliminates the disease, various drugs are used to control the complaints. Treatment is determined by the way the disease affects the body. Exercise , skin care and healthy nutrition are areas that should be considered for the protection of health.

Various drug treatments are available to reduce complaints such as Raynaud’s phenomenon, gastric reflux, difficulty in swallowing, diarrhea, constipation, and joint pain. Anti-hypertensive treatment is given if high blood pressure occurs. Pulmonary hypertension is a rare but serious complication. For this reason, lung and heart functions are checked at regular intervals and drug therapy is started if necessary.

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