Nursing Assessment, Management and Treatment of Autoimmune Diseases
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Systemic scleroderma (systemic sclerosis) Systemic scleroderma is characterized by changes in connective tissue that oc- cur in many parts of the body. Systemic sclerosis can involve the skin, esopha- gus, gastrointestinal tract, lungs, kidneys, heart, and other internal organs. The dis- ease can also affect blood vessels, mus- cles, and joints (Scleroderma Foundation, 2021). Affected tissues become hard and fi - brous, leading to functional impairment. There are two major patterns that system- ic scleroderma can take-- diffuse or lim- ited patterns (Scleroderma Foundation, 2021). ● Diffuse scleroderma : In diffuse scleroderma thickening of the skin occurs at a rapid rate and involves more areas of the skin than the limited disease. People with diffuse scleroderma are at higher risk of developing sclerosis or fibrous hardening of the internal organs. ● Limited scleroderma : Limited scleroderma affects about 50% of persons who have scleroderma. It progresses more slowly and is a more benign illness than diffuse scleroderma. Internal issues may evolve, but they are typically less frequent and less severe compared to diffuse scleroderma. However, patients with limited scleroderma can develop pulmonary hypertension, which causes a narrowing of the blood vessels of the lungs, impaired blood flow to the lungs, and shortness of breath.
Risk factors Several factors may influence the risk of developing scleroderma. These include the following (Mayo Clinic, 2019): ● Genetics : It is possible that gene variations may be a risk factor for the development of scleroderma. A small number of cases of scleroderma seem to run in families. The disease also appears more often in certain ethnic groups. For example, Choctaw Native Americans are more likely to develop scleroderma that affects the internal organs of the body. ● Environmental triggers : Research findings indicate that scleroderma symptoms may be triggered by exposure to some viruses, medications, or drugs. Work exposure to harmful chemicals may also increase the risk of scleroderma development. ● Immune system issues : As an autoimmune disease, the body’s immune system negatively impacts its own connective tissues. In about 15% to 20% of cases, someone who has scleroderma also has symptoms of another autoimmune disease such as lupus or rheumatoid arthritis. Complications Scleroderma complications range from mild to severe. These include the follow- ing (Mayo Clinic, 2019): ● Raynaud’s disease : A form of Raynaud’s disease sometimes occurs with systemic scleroderma. Raynaud’s disease in these patients can be so severe that impaired blood flow permanently damages fingertip tissue, leading to pits and/or skin sores. In some patients, fingertip tissue may die and amputation may be necessary.
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