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Systemic sclerosis is a rare autoimmune disease affecting approximately 2,5 million people worldwide. The disease occurs 4 times more often in women, often still professionally active and caring for children.
What is systemic sclerosis?
Systemic scleroderma, or scleroderma, is a rare autoimmune disease that affects the connective tissue of the patient. The name of the disease is derived from the Greek words: sclera meaning hard as well dermatologists at dermawhich is the skin. Due to the involvement of internal organs in the course of the disease, it has been described as systemic scleroderma. It is worth emphasizing that scleroderma is typical for thickening and scarring of the epidermis, as well as other tissues. This is due to the excessive accumulation of collagen.
The occurrence of systemic scleroderma in the world varies greatly. In Poland, about 2018 thousand cases were recorded in 5,5. The prognosis for this autoimmune disease is not favorable for patients. The 5-year survival from the diagnosis is 74,9% on average, while the 10-year survival is 62,5%. It should be remembered that the highest mortality is observed in scleroderma with lung involvement.
Women’s disease?
Systemic sclerosis is much more common in women than in men. In most cases, it affects people between 25 and 55 years of age. It is still unknown what exactly causes the development of the disease. However, numerous scientific studies indicate that these factors can be many, including genetic, immunological and hormonal background.
Symptoms of systemic scleroderma
Systemic sclerosis is a disease that “takes your face”. It causes characteristic changes on the patient’s face. Pathological changes are also observed in the area of internal organs (e.g. lungs, heart or kidneys) and even blood vessels which, due to fibrosis, are unable to perform their functions.
In the course of scleroderma, interstitial lung disease may develop. It occurs in 1 in 4 people within three years of diagnosis of systemic sclerosis and is the leading cause of death among these patients. Lung tissue then becomes fibrotic, so that less and less oxygen reaches the lungs. The clinical symptoms of the disease are quite nonspecific. The patient complains of shortness of breath (e.g. during exercise) and coughing. Interstitial lung lesions are diagnosed more often in women around 50 years of age. The exact etiology and pathogenesis is still unknown.
Diagnosis and treatment of systemic scleroderma in Poland
Systemic sclerosis is an incurable disease. That is why fast and proper diagnostics is so important. It is recommended that it take place in reference centers with appropriate experience, with the participation of interdisciplinary teams of specialists.
The main goal of treatment is to improve the patient’s quality of life. Unfortunately, no drug has yet been developed that could reverse the disease, but there are some that can slow its progression. For this purpose, it is best to consult and follow the recommendations of a physician who has specialist knowledge of the disease.
More information on interstitial lung disease in the course of systemic sclerosis can be found at: www.plucapolski.pl