Progressive systemic sclerosis is an autoimmune disease that causes skin, joint, and internal organ problems due to an overproduction of collagen. It has no known cure and is suspected to have genetic and environmental causes. Symptoms include skin changes, joint pain, and breathing difficulties. Treatment is individualized and includes medication and physical therapy.
Progressive systemic sclerosis is an autoimmune disease that causes problems with the skin, joints and internal organs. The defining feature of the disease is an overproduction of collagen, a type of connective tissue, in the body. When there is too much collagen, areas of skin thicken and harden, joints become less mobile, and vital blood vessels are damaged. People who have this condition may experience a variety of symptoms that gradually worsen over time, possibly leading to permanent disability or life-threatening heart and lung conditions. Medications and physical therapy help many patients find relief from their symptoms, but there is currently no known cure for the disorder.
The causes of progressive systemic sclerosis are not fully understood. Genetic factors are suspected to play a role, but no clear genetic link has been discovered. Long-term exposure to environmental toxins, such as silica dust, pesticides, and epoxy glues, is a common finding among patients. The disease occurs most frequently in adult women in their 40s.
In many cases, skin changes are the first noticeable symptoms of progressive systemic sclerosis. Constriction of blood vessels in the fingers and toes can cause them to turn blue and lose sensation when exposed to even moderate cold, a condition called Raynaud’s phenomenon. The skin on the fingers and toes can also become very thick, and the nails can become brittle and break. Excess calcium and collagen in your finger joints could cause your fingers to bend awkwardly and also lose dexterity. Itchy patches on the skin and leg ulcers are also common.
The disease can also cause pain and stiffness in major joints such as the knees, elbows and hips. Digestive problems are common, such as heartburn, frequent nausea, and vomiting. If the blood vessels in the lungs are damaged and scarred, a person may have difficulty breathing and develop very high blood pressure. Heart failure is also possible if the disease causes inflammation and hardening of the tissues around the organ.
A team of physicians, including dermatologists and internists, is usually involved in the diagnosis of progressive systemic sclerosis. Physical exams that reveal major skin changes usually provide the first clues that the disorder exists. X-rays and other diagnostic imaging scans are useful for revealing the presence of excess collagen and measuring the extent of damage to blood vessels and internal organs. Blood samples are also tested to confirm the presence of abnormal antibodies and other characteristic signs of sclerosis.
Treatment for progressive systemic sclerosis is highly individualized and geared towards improving each patient’s particular symptoms. Skin problems are usually treated with topical corticosteroids and moisturizers. Patients with Raynaud’s phenomenon may need to avoid venturing outdoors during very hot or cold weather to prevent complications. Oral steroids are often given for joint problems and blood pressure stabilizers, heartburn medications and bronchodilators can help with other symptoms. Regular exercise or guided physical therapy is important for staying mobile and independent in the later stages of the disease.
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