A. A healthy, young patient of mine once asked me what the chances were that she might one day develop a "terrible disease." When I asked her what she meant by "terrible disease," she surprised me: she didn't say a disease that could be fatal, but rather a disease that could attack every part of her body. By that definition, systemic lupus erythematosus (lupus for short) is, indeed, a terrible disease.

The cause of lupus remains unknown, but there is solid evidence that genetics, epigenetics (changes in chromosomes that affect gene activity), environmental factors, viruses and infections play a role. Further study of these variables is expected to improve our understanding of causes, which should lead to improved diagnosis, prognosis, prevention, and treatment.

The most serious health risks are cardiovascular disease, kidney disease and stroke. Specifically, people with lupus are at increased risk for atherosclerosis (hardening of the arteries). In some people, inflammation can occur in the heart itself (myocarditis and endocarditis) or the membrane that surrounds it. Endocarditis can damage heart valves, which can result in heart murmurs. When the disease affects the kidneys, patients generally require intensive drug treatment to prevent permanent damage. Lupus also may attack the brain or central nervous system, which can cause seizures or stroke.
The most serious health risks are cardiovascular disease, kidney disease and stroke. Specifically, people with lupus are at increased risk for atherosclerosis (hardening of the arteries). In some people, inflammation can occur in the heart itself (myocarditis and endocarditis) or the membrane that surrounds it. Endocarditis can damage heart valves, which can result in heart murmurs. When the disease affects the kidneys, patients generally require intensive drug treatment to prevent permanent damage. Lupus also may attack the brain or central nervous system, which can cause seizures or stroke.
García-Carrasco M1, Mendoza-Pinto C, Cardiel MH, Méndez-Martínez S, García-Villaseñor A, Jiménez-Hernández C, Alonso-García NE, Briones-Rojas R, Ramos-Álvarez G, López-Colombo A. "Health related quality of life in Mexican women with systemic lupus erythematosus: a descriptive study using SF-36 and LupusQoL(C)." Lupus 21.11 Oct. 2012. .
Contraception and family planning are important considerations given the risks of disease flare with exogenous estrogens and pregnancy and with the teratogenic risks of some SLE drugs. Estrogen therapies have typically been avoided to prevent disease flares; progesterone-only contraception is more often considered. [144] However, studies have suggested that oral estrogen-containing contraceptives may not be associated with disease flares or thrombosis risk in patients with mild lupus without antiphospholipid antibodies. [52, 145]
The body’s tolerance of the antigens present on its own cells, i.e., autoantigens or self-antigens. It is theorized that autoreactive T lymphocytes are destroyed in the thymus by negative selection or in peripheral blood. Autoreactive T cells that escape destruction in the thymus may become tolerant because they are exposed to thousands of autoantigens as they circulate in the blood.
Numerous studies suggest that moderate intake of alcohol may decrease the risks of developing cardiovascular disease problems, increase HDL good cholesterol levels, and may even decrease the risk for certain cancers. However, the sugar it contains will increase your calorie consumption (potentially contributing to weight gain) and regular alcohol consumption may increase the risk for breast cancer.
Patients with SLE should be educated to avoid triggers for flare. Persons with SLE should avoid ultraviolet light and sun exposure to minimize worsening of symptoms from photosensitivity. Diet modification should be based on the disease activity. A balanced diet is important, but patients with SLE and hyperlipidemia, for example, should be placed on a low-fat diet. Many patients with SLE have low levels of vitamin D because of less sun exposure; therefore, these patients should take vitamin D supplements. Exercise is important in SLE patients to avoid rapid muscle loss, bone demineralization, and fatigue. Smoking should also be avoided.
The severity of lupus varies from mild to life threatening. Kidney problems and neurologic complications are more dangerous than the rashes, arthritis or other symptoms. After many years of having lupus, patients may develop hypertension (high blood pressure), accelerated atherosclerosis (plaque and fat build-up in the arteries), heart and lung diseases, kidney failure or osteoporosis. With proper treatment, the majority of people diagnosed with lupus have a normal life expectancy, but many will experience disabilities. Each patient will likely have his or her own specific pattern of symptoms and flares, but the disease can change over time.
The lupus erythematosus (LE) cell test was commonly used for diagnosis, but it is no longer used because the LE cells are only found in 50–75% of SLE cases, and they are also found in some people with rheumatoid arthritis, scleroderma, and drug sensitivities. Because of this, the LE cell test is now performed only rarely and is mostly of historical significance.[72]
Normally, our immune system produces proteins called antibodies that protect the body from these foreign invaders. When you have lupus, your immune system cannot tell the difference between these foreign invaders and your body’s healthy tissues, so autoantibodies are made that damage and destroy healthy tissue (auto means self and anti means against, so autoantibody means against self). These autoantibodies cause inflammation, pain, and damage in various parts of the body.

An abnormal elevation of temperature. The normal temperature taken orally ranges from about 97.6° to 99.6°F (36.3°C to 37.6°C). Rectal temperature is 0.5° to 1.0°F higher than oral temperature. Normal temperature fluctuates during the day and is lowest in the morning and highest in the late afternoon; these variations are maintained during a fever. The expended basal energy is estimated to be increased about 12% for each degree centigrade of fever.

A substance (generally a protein, polypeptide, or peptide) that stimulates the differentiation, division, development, and maintenance of cells and the tissues they make up. Growth factors are signaling molecules released by certain groups of cells, e.g., lymphocytes, to influence the activities of other cells. Growth factors can be divided into families, e.g., platelet-derived GFs, transforming GFs, and angiogenic GFs. They are released normally during fetal and embryonic development, wound healing, and tissue maturation. Massive releases of GFs are characteristic of some types of cancer cells. Artificial GFs, e.g., granulocyte colony-stimulating factor, are used in health care to restore depressed levels of cells to normal values, e.g., in patients who have received chemotherapy.
The panel judged the effect of extended AC as a large benefit, reducing VTD with increase in bleeding risk as a moderate harm. For the comparisons of different AC intensities, the panel decided to use the evidence from observational studies because it judged that it probably better reflects reality given that the randomised controlled trials (RCT) are severely flawed (indirectness of intervention as most patients did not reach the INR >3 goal). They judged the reduction in VTD as a large benefit and the bleeding increase as a large harm. Hence, the panel considered that the balance could favour the intervention only when the risk of VTD recurrence is particularly high.
Other tests for lupus depend on the symptoms patients are experiencing, says Kaplan. For example, chest X-rays and echocardiograms may be necessary to investigate fluid around the lungs and the heart. If doctors suspect nephritis is present, the patient may need a kidney biopsy. Early diagnosis and treatment can help to avoid complications, he adds.

Blood clots are seen with increased frequency in lupus. Clots often occur in the legs (a vein clot, called deep venous thrombosis), lungs (a lung clot, called pulmonary embolus), or brain (stroke). Blood clots that develop in lupus patients may be associated with the production of antiphospholipid antibodies. These antibodies are abnormal proteins that may increase the tendency of the blood to clot.
The role of the immune system in causing diseases is becoming better understood through research. This knowledge will be applied to design safer and more effective treatment methods. For example, completely revising the immune system of people with extremely aggressive treatments that virtually temporarily wipe out the immune system is being evaluated. Current studies involve immune eradication with or without replacement of cells that can reestablish the immune system (stem-cell transplantation).
Toll-like receptors (TLRs) are an essential arm of the innate immune response to bacteria, viruses and fungi and link recognition of distinct features of these microbes to the induction of pro-inflammatory signaling pathways. These receptors are able to respond to broad classes of pathogens because each TLR recognizes specific conserved microbial features.
The lupus erythematosus (LE) cell test was commonly used for diagnosis, but it is no longer used because the LE cells are only found in 50–75% of SLE cases, and they are also found in some people with rheumatoid arthritis, scleroderma, and drug sensitivities. Because of this, the LE cell test is now performed only rarely and is mostly of historical significance.[72]
SLE is an autoimmune disease involving multiple organ systems, a clinical pattern of flares and remissions, and the presence of anti-nuclear autoantibodies. Whereas early symptoms most frequently involve the skin and joints, disease morbidity and mortality are usually associated with cardiovascular events and damage to major organs, particularly the kidneys. Many of the current therapeutic options are considered to be inadequate because of toxicities, accrual of organ damage, and insufficient control of the underlying disease pathology. Improved understanding of SLE pathogenesis and immunology has led to the identification of new treatment targets. Current interest is mainly focused on the targeted immunosuppressive actions provided by biologic therapy. Although the potential long-term beneficial or harmful effects of the new molecular treatments are unclear, their precise molecular targeting may reveal key relationships within the immune system and advance the cause of individualized molecular medicine.
Studies have shown that lupus multiplies a woman’s risk for osteoporosis five times4 – an alarming figure, no doubt. Osteoporosis is a condition where bone density decreases, leading to an increased risk of skeletal fracture at older age. Like lupus, osteoporosis has no cure, so it's a condition better prevented  than treated, especially for women.
Why the test is used: Abnormalities in blood cell counts, including white blood cells and red blood cells, may occur in people with lupus. This may be related to the lupus, lupus treatments, or infection. For example, leukopenia, a decrease in the number of white blood cells, is found in about 50% of people with lupus. Thrombocytopenia, or a low platelet count, occurs in about 50% of people with lupus, as well. Doctors can use this test to monitor these potentially serious problems.

In addition to helping with lupus nephritis, these drugs may be prescribed to reduce inflammation of the heart and the lining surrounding the lungs. Disease-modifying antirheumatic drugs (DMARDs) used for rheumatoid arthritis treatment, such as methotrexate, may be an effective and well-tolerated option for reducing swelling in patients with severe arthritis, adds Caricchio. DMARDs are another type of immunosuppressant.

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