What is Systemic Sclerosis (Scleroderma)?

Systemic sclerosis (scleroderma) is an autoimmune rheumatic disease in the group of collagen tissue diseases. It is seen more in women than men. Genetic causes, microchimerism, X chromosome inactivation, environmental factors, various chemicals and drugs are held responsible for the emergence of the disease. However, in general, the etiopathogenesis mechanisms are not clear, as in all rheumatic diseases, and medical studies continue in this regard.

What are the signs of scleroderma?

In general, an increase in fibrosis is observed in the collagen tissue (connective tissue), which serves as the body’s support tissue. The most noticeable finding of this increase is the hardening of the skin. Hardening is noted on the fingers, back of the hand, forearm, trunk, scalp and scalp. While there is widespread skin hardening in the whole body, which we sometimes call diffuse; and sometimes localized hardening can be seen in certain areas such as plaque or band. Skin hardening is the easiest finding for doctors to catch and follow. Rank can be determined by Rodnan Leather Score (RSS). However, if the patient cannot be treated at a very advanced stage, the skin may appear to be softened due to the atrophy of the subcutaneous tissue. When this situation develops, fibrotic changes have already started in the internal organs and even settled.

The most affected organ in internal organ involvement is the lungs. Inflammatory tissue, which starts from the alveolar distance in the lungs, where gas exchange takes place, may become fibrotic over time, and oxygenation is impaired as gas exchange is impaired, and it causes the development of pulmonary hypertension (lung hypertension) by affecting the heart, which is different from systemic hypertension and is more difficult to treat. . The heart may fail due to this stress, and patients may be candidates for heart-lung transplantation in the long run if left untreated. In addition, conduction defects in the heart, impaired electrolyte exchange in the kidneys, and sudden and fatal increase in systemic hypertension, and the development of scleroderma renal crisis can be seen. Also, liver and biliary tract, pancreas, nervous system, esophagus, stomach and intestines, in short, all organs and systems are affected by the disease.

Hardening of the skin can lead to disruption of nutrition of the extremities and the opening of gangrenous wounds, especially on the tips of the hands and feet, and the development of auto-amputation. Again, excessive hardening of the skin over the joint can lead to joint contractures and disability. Common calcifications (petrifications) can be seen in the skin, which we call ectopic, especially in the contact areas, and the body tries to remove these calcifications from time to time, and in this case, it can cause skin inflammation.

What are the treatments for Scleroderma Patients?

Since this increase in fibrosis in the connective tissue also develops by autoimmune mechanisms, agents that regulate the disease with immunosuppressive properties and cortisone are used in the treatment, as we mentioned before. Separate vasodilating agents may also be given to lower the pulmonary pressure. The important thing is for patients to reach internal medicine-rheumatology specialists, to map the systemic involvement after diagnosis and to make a treatment plan according to this map.

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