Systemic sclerosis (scleroderma) is an autoimmune rheumatic disease in the group of collagen tissue diseases. It is more common 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, as in all rheumatic diseases, the etiopathogenesis mechanisms are not clear, and medical studies continue on this subject.
What are the findings of scleroderma?
In general, the supporting tissue of the body There is an increase in fibrosis in the collagen tissue (connective tissue) that serves as a function. The most noticeable finding of this increase is the hardening of the skin. Hardening of the fingers, back of the hands, forearm, trunk, skull and scalp is noted. While there is widespread skin hardening throughout the body, which we sometimes call diffuse; Sometimes, hardening may occur in certain areas (localized) such as plaques or bands. Skin hardening is also the easiest symptom for doctors to detect and follow. Its degree can be determined by Rodnan Skin Score (RSS). However, if the patient is not treated at a very advanced stage, the skin may appear to have softened due to atrophy of the subcutaneous tissue. When this situation develops, fibrotic changes have already begun and even become established in the internal organs.
The organ most affected by internal organ involvement is the lungs. Inflammatory tissue that starts in the alveolar space where gas exchange occurs in the lungs may become fibrotic over time, and oxygenation is impaired as gas exchange is disrupted and causes the development of pulmonary hypertension by affecting the heart, which is different from systemic hypertension and more difficult to treat. . The heart may fail due to this stress, and if patients are not treated, they may become candidates for heart-lung transplantation in the long term. Conduction defects in the heart, impaired electrolyte exchange in the kidneys, sudden and fatal increase in systemic hypertension and scleroderma renal crisis may develop. In addition, the liver and bile ducts, pancreas, nervous system, esophagus, stomach and intestines, in short, all organs and systems are affected by the disease.
Skin hardening causes deterioration in the nutrition of the extremities and causes gangrenous wounds, especially on the extremities of the hands and feet. It may cause death and auto-amputation. Again, excessive hardening of the skin over the joint can lead to joint contractures and disability. Common calcifications, which we call ectopic, can be seen on the skin, especially in contact areas, and the body tries to remove these calcifications from time to time, which may cause skin inflammation.
What is the treatment of Scleroderma Patients?
Since this increase in fibrosis in the connective tissue develops through autoimmune mechanisms, immunosuppressive disease-regulating agents and cortisone are used in the treatment, as we mentioned before. Separate vasodilator agents may also be given to reduce pulmonary blood pressure. The important thing is for patients to reach internal medicine-rheumatology specialists, to map the systemic involvement after diagnosis, to make a treatment plan in accordance with this map, to ensure that the patients comply with the treatment very well, and to have regular check-ups.
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