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Systemic scleroderma (e.g. scleroderma, SSC) is a chronic autoimmune disease of connective tissue. The disease affects the skin, internal organs and blood vessels. Scleroderma causes fibrosis of the skin and internal organs, which in turn leads to their damage. Find out what are the causes and symptoms of systemic scleroderma.
Systemic scleroderma – characteristics
Systemic sclerosis (SSc) is one of the autoimmune diseases that affects connective tissue cells. It is a rare, chronic disease with a heterogeneous course. It is characterized by progressive fibrosis of the skin, blood vessels and internal organs: kidneys, lungs, heart, and gastrointestinal tract. The blood supply to the organ tissues drops, which damages their structure and disrupts their functioning. It is estimated that the annual incidence in the world is between 4-12 people per million. About 10 people are ill in Poland. Systemic scleroderma affects women more often than men. More than 80% of patients are women. The greatest number of cases occurs between 30 and 50 years of age. There are also cases in children.
Systemic sclerosis – causes
The causes of systemic sclerosis are still not fully understood. Some studies indicate a genetic, autoimmune and hormonal background. They suggest that the disease develops more often in people with family history of autoimmune and hormonal diseases. Other studies show the possible influence of external factors, especially harmful substances. Among them there are: benzene, xylene, vinyl chloride, silicone, toluene, silicon.
Systemic scleroderma – symptoms
Scleroderma is characterized by thickening, hardening of the skin. The next stage is the loss of elasticity, which leads to fibrosis. The same process affects internal organs and blood vessels. Damaged vessels do not fulfill their function because they are not able to bring the right amount of blood to the organs. As a result, the organs are damaged, both by the process of fibrosis, scarring and a decrease in blood supply. The patient is accompanied by severe pain in joints and muscles, especially in the hands and knees. There are also other ailments, depending on which internal organs are attacked by systemic scleroderma.
Systemic scleroderma – local scleroderma
Scleroderma can take many forms, from mild to severe, depending on where it occurs. And so, local scleroderma is characterized by the fact that the lesions mainly affect the skin and the closest tissues (i.e. fat, muscle, less often bone). Contractures in the joints may occur depending on the depth of the scarring lesions. A common symptom of local scleroderma is a scar on the scalp that resembles a scar from a saber cut.
Systemic sclerosis – types
We can distinguish two forms of scleroderma: the limited form and the generalized form.
Limited systemic sclerosis – the disease develops slowly but continuously. Often the first symptom of the disease is Raynaud’s phenomenon, i.e. paroxysmal spasm of the blood vessels in the fingers, most often caused by cold or stress. Then there are skin lesions, hardening of connective tissue, skin and subcutaneous tissue as well as deep tissues. This form of scleroderma does not affect internal organs. The patient experiences contractures and pain in the joints as well as muscle weakness. The patient is suffering from ischemia of the fingers, pale skin, numbness and a tingling sensation, as well as pain. The symptoms of scleroderma can be confused with RA, but they can both occur at the same time. After many years, this form of the disease may develop problems with the esophagus, pulmonary hypertension or cirrhosis of the liver.
Systemic systemic sclerosis – this form has a more intense and rapid course. Skin changes develop rapidly. The advanced form develops in 3-6 years. Skin lesions affect the hands, arms, thighs, and torso. At the same time, the patient notices the appearance of Raynaud’s phenomenon. The disease also attacks internal organs: lungs, heart, kidneys and gastrointestinal tract.
Systemic sclerosis – treatment
Systemic sclerosis is an incurable disease. The lesions are irreversible. Treatment of systemic scleroderma is based on relieving the symptoms of the disease and preventing complications. The patient is treated by a rheumatologist. The patient is given painkillers and drugs that inhibit the development of systemic scleroderma. Symptoms of internal organs are also alleviated. Raynaud’s symptoms are treated with drugs that dilate the blood vessels.
Physical therapy and exercise are recommended to improve mobility. The sick person must also eliminate the factors that cause vasospasm (smoking, cold, stress).
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