Systemic Lupus Erythematosus
Like many autoimmune diseases, it is difficult to determine why it develops, but it is often due to genetics and the environment. Susceptibility genes plus environmental risk factors (for example a sunburn), cause damage to cell DNA, causing cell death, exposing the inside of the cell (including parts of the nucleus, such as DNA, histones, and other proteins) to the rest of the body. Because the individual is more susceptible, their immune cells are more likely to think these parts of the cell are foreign or antigens, and they attack them. The body has a buildup of these nuclear antigens, and the body forms antinuclear antibodies that attach themselves to the antigens. These antigen-antibody complexes enter the bloodstream and get deposited in blood vessel walls, organs, and tissues, such as the skin, kidneys, heart, and joints – which cause inflammation. When this tissue damage occurs, it is known as a Type III hypersensitivity reaction. Environmental triggers include UV radiation, viruses, bacteria, sex hormones (such as estrogen), smoking, and medications (such as procainamide, hydralazine, or isoniazid). Lupus is about 10 times more common in women during reproductive years than men.
The inflammation is what causes the symptoms seen in lupus. Diagnosis is difficult because the symptoms vary greatly between individuals. There are the typical general symptoms, such as fever, joint pain, rash, and weight loss; and specific symptoms that depend on what organ or tissue is being affected. The American College of Rheumatology has developed criteria for the diagnosis of SLE, which uses a point system based on the severity of the symptom. Symptoms include fever; skin related symptoms, such as a red butterfly rash (also called malar rash) on the face; a discoid rash, which is a plaque-like red patch that can scar; photosensitivity, which causes other rashes from sun exposure; nonscarring alopecia; and ulcers in the mucous membrane of the mouth or nose. The main joint related symptom is arthritis, or inflammation of 2 or more joints and 30 min of morning stiffness. Symptoms in the serosa, or outer membrane of tissues, may include serositis, or inflammation of the serous membrane around the lungs, heart, or other organ. Neurologic symptoms include migraine headaches, seizures, stroke, and psychosis. Symptoms related to the blood depend on the cells being attacked and include anemia, when RBCs are being attacked; leukopenia, WBCs are being attacked; or thrombocytopenia, when platelets are being attacked. Symptoms affecting the kidneys include proteinuria, nephritis, and glomerulonephritis. An immunologic disorder is seen with a positive finding of antibodies in the blood, including antinuclear antibodies or other autoantibodies. Other symptoms often seen with SLE include Raynaud phenomenon, when fingers and toes turn very pale or bluish when exposed to cold; also nausea, diarrhea, abdominal pain, liver damage, pancreatitis, and repeated miscarriages in obstetrical patients. Symptoms of lupus are exhibited by flare ups and then periods of remittance. It is important to teach patients the need for preventing flare-ups by doing things such as avoiding sun exposure, proper skin care, avoiding sources of infection, and reducing stress. Surgery and pregnancy can also trigger flare-ups. In most cases, if the initial inflammation is controlled, the long-term prognosis is good.
SLE should be classified as mild or severe – mild symptoms including fever, arthritis, headache, rash; and severe symptoms including hemolytic anemia, large pleural and pericardial involvement, significant renal damage, and complicated CNS involvement, among others. The most common presenting symptoms in mild SLE include arthritis and arthralgia, and can usually be controlled with NSAIDs. Regardless of disease severity, the antimalarial drug hydroxychloroquine is indicated for all SLE patients because it decreases flares and mortality. In severe disease, corticosteroids and immunosuppressants are given to limit the immune response. Chronic disease should be treated with the lowest dose of corticosteroids and anti-inflammatories that will maintain remission. Treatment is also based on the patient’s presenting clinical symptoms.
During flare-ups, patients with SLE are acutely ill, and the nurse focuses care on the symptoms presented. The nurse should monitor for renal dysfunction and the effects of medications. Neurologic status must be frequently assessed for the development of cognitive dysfunction resulting from the disease process or steroid therapy.
Ok, let’s go over a few questions to review.
A 30-year-old woman has been diagnosed with systemic lupus erythematosus (SLE) and would like to become pregnant. When teaching the patient about the disease, the nurse should include which of the following? (Choose all that apply)
- SLE is a hereditary disorder of women that usually shows clinical symptoms when a woman becomes pregnant.
- SLE is a disease that causes an inflammatory response because antibodies bind with estrogen receptors.
- SLE is an autoimmune disorder that has an extremely variable course, with no way to predict its progression.
- SLE often causes miscarriages, therefore she should consult a high risk obstetrician to carefully consider the safety of medications used for treatment during pregnancy.
If you chose #3 and 4, you’re correct! Women are more at risk for SLE, but it is not confined just to women, and clinical symptoms may worsen during pregnancy, but are also evident prior to pregnancy.
A nurse is taking a health assessment of a patient who complains of low grade fever, fatigue, joint pain, muscle pain, and photosensitivity. SLE is suspected, and the nurse would check for which of the following signs that also occurs with SLE?
- Red butterfly rash across cheeks and nose
If you chose #3, you’re right! A red butterfly rash across the face is a classic sign of SLE.
The pathophysiology of SLE involves
- Overproduction of collagen that affects internal organs
- The body producing various autoantibodies that attack parts of the cell nucleus
- Abnormal IgG complexes that cause inflammation
- Immunodeficiency caused by increased activity of T-cells and decreased activity of B-cells.
If you chose #2, you’re correct! In SLE, the body produces many autoantibodies that are directed against parts of the cell nucleus, particularly DNA. Antigen-antibody complexes form and enter the bloodstream, getting deposited in blood vessel walls, organs, and tissues – which causes inflammation.
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