Systemic sclerosis is a connective tissue disease that most often affects the skin, gastrointestinal tract, lungs, kidneys and cardiovascular system. If there is an extensive involvement of the skin, the disease can be called scleroderma. Patients with diffuse scleroderma have widespread fibrosis of the skin and the kidneys, lungs and heart are frequently compromised, presenting a much worse prognosis.
Systemic sclerosis is characterized by widespread fibrosis, microvascular abnormalities and numerous immunological defects. The fibrosis probably represents the final state of the disease.
The early phase of skin involvement is characterized by inflammation and edema. Inflammation occurs most frequently in the lower dermis and subcutaneous tissue, with a cellular infiltration of lymphocytes, plasma cells and histiocytic cells.
The Raynaud phenomenon occurs in 90% of the patients with systemic sclerosis and is due to the vasospasm of the small digital arteries. Structural abnormalities of the vessel wall may or may not occur. In systemic sclerosis, spontaneous or cold-induced vasoconstriction can be an important cause of dysfunction of all compromised organs. For example, the flow of cortical blood in the kidney decreases considerably after a cold pressure test. Some of the pulmonary function abnormalities observed in this disorder may be due to a similar vasoconstriction.
The ability of the coronary arteries to vascularize is markedly impaired in patients with systemic sclerosis, presumably due to the structural abnormalities of the small coronary vessels.
The identification of immunological, vascular and fibroblastic abnormalities in systemic sclerosis has given rise to several new therapeutic approaches.
Although there is no cure for systemic sclerosis, much can be done to improve the quality of life of the patient. The perception of systemic sclerosis as a rapidly progressive disease is often incorrect, since many patients have long periods of remission of the disease, which sometimes last for years.
Systemic sclerosis is predominantly a disease of middle age and is three times more common in women than in men. In almost all cases the skin is compromised.
It is extremely important that the patient is attended by a doctor who has experience with the disease and is aware of the need to assess visceral involvement, especially the gastrointestinal tract, lungs, heart and kidneys. It is important that the patient who smokes stop doing so, since nicotine is a potent vasoconstrictor that exacerbates the symptoms and signs of the disease. The blood pressure of patients with systemic sclerosis should be controlled frequently, since hypertension is a manifestation of renal compromise, and malignant hypertension, which can occur abruptly, is the main cause of death in this disease. That is, early treatment to control hypertension can save lives.
Why can patients with Sclerosis be treated in a hyperbaric chamber?
The action of hyperbaric oxygen dilutes high doses of O2 in the blood plasma, being rapidly used by the cells and reaching tissues where the perfusion is compromised. This achieves greater efficiency in the desinflammatory process and the regeneration of tissues in rheumatic diseases, such as systemic sclerosis.
World Health Organization.
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