21/8-2017 
 
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Connective Tissue

Scleroderma

Definition and causes of Scleroderma

Scleroderma means hard skin, and is a connective tissue disease, where especially the small blood vessels (capillaries) are damaged as a result of an autoimmune reaction.

Auto immune diseases are a group of diseases where the body's own immune system starts to attack the body itself. The reason for this reaction is unknown. As a result of damage to the capillaries and the subsequent lack of oxygen the surrounding tissue is damaged. Changes occur, especially seen on the skin of the face, arms and legs.

The disease is rare, affects 3 times as many women as men aged 30-50 years.
 

Symptoms of scleroderma

The symptoms start with long-standing tendency to Raynaud's phenomenon, which is brief spasm of the small blood vessels in the fingers and toes, which manifests itself as blue discoloration or paleness of the skin when subjected to cold.

Slowly skin changes develop, which typically appears on the fingers and toes first. The initial changes are swollen or sausage fingers and after weeks or few months, the development of thickening of the skin, experienced as smooth, hard and leather-like . Thickening of the skin of the face, can give a mask-like expression with a pointed nose and small mouth.

In most patients changes to the lungs in the form of stiff lungs develops, which often produces no symptoms. However, shortness of breath and coughing may be seen.

In some patients changes in the esophagus can lead to symptoms such as acid regurgitation and a burning sensation behind the sternum.
 

Precautions and diagnosis

A blood test can reveal whether you have elevated levels of certain antibodies in the blood, often associated with scleroderma. An X-ray can reveal whether there is involvement of the lungs or possibly esophagus.
 

Treatment of scleroderma

The treatment of scleroderma includes various drugs that can slow down the damage to the body's connective tissue, and drugs to that relieve symptoms, or reduce the risk of developing complications.
 

Prognosis and complications

The disease course is highly variable. In cases where the disease is widespread and severe, the prognosis is often poor and can result in severe disability and increased mortality. In other cases, with less severe disease the prognosis is good, and development of life-threatening complications rare. Overall, the mortality rate is only slightly elevated compared to the background population.

Serious complications may include kidney failure and stiff lungs sometimes associated with cardiac impact, which also are the most frequent causes of death that are directly linked to scleroderma.

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