In addition to hormonal mechanisms, specific genetic influences found on the X chromosome may also contribute to the development of SLE. Studies indicate that the X chromosome can determine the levels of sex hormones. A study has shown an association between Klinefelter syndrome and SLE. XXY males with SLE have an abnormal X–Y translocation resulting in the partial triplication of the PAR1 gene region.
Disease-modifying antirheumatic drugs (DMARDs) are used preventively to reduce the incidence of flares, the progress of the disease, and the need for steroid use; when flares occur, they are treated with corticosteroids. DMARDs commonly in use are antimalarials such as hydroxychloroquine and immunosuppressants (e.g. methotrexate and azathioprine). Hydroxychloroquine is an FDA-approved antimalarial used for constitutional, cutaneous, and articular manifestations. Hydroxychloroquine has relatively few side effects, and there is evidence that it improves survival among people who have SLE. Cyclophosphamide is used for severe glomerulonephritis or other organ-damaging complications. Mycophenolic acid is also used for treatment of lupus nephritis, but it is not FDA-approved for this indication, and FDA is investigating reports that it may be associated with birth defects when used by pregnant women.
A group of people who review, approve, and monitor the clinical study protocol. Their role is to protect the rights and welfare of human research subjects participating in a study. The group typically includes people with varying backgrounds, including a community member, to make sure that research activities conducted by an organization are completely and adequately reviewed. Also known as an institutional review board (IRB) or ethics committee.
Systemic sclerosis (SSc): Similar symptoms between SSc and lupus are reflux and Raynaud's disease (when your fingers turn blue or white with cold). One difference between SSc and lupus is that anti-double-stranded DNA (dsDNA) and anti-Smith (Sm) antibodies, which are linked to lupus, don't usually occur in SSc. Another differentiator is that people with SSc often have antibodies to an antigen called Scl-70 (topoisomerase I) or antibodies to centromere proteins.
The clinical manifestations of systemic lupus erythematosus are fundamentally the same in children and adults.15 In two descriptive studies25,26 of children with the disease, the most frequent manifestations were fever, rash, arthritis, alopecia, and renal involvement. Compared with adults, children have a higher incidence of malar rash, anemia, leukocytopenia,27 and severe manifestations such as neurologic or renal involvement.28
B cells obtain help from T cells in the antibody response by acting as antigen-specific antigen presenting cells. A direct signal through binding of antigen to membrane Ig can enhance B cell antigen presentation and T-dependent B cell activation, but is not required for a productive interaction between a small resting B cell and a differentiated helper T cell.
Lupus is a chronic autoimmune disease that causes the immune system to attack healthy tissue resulting in inflammation, particularly of the skin, joints, kidneys, heart, lungs, blood vessels and brain. It develops most commonly in women between the ages of 15-45, and occurs more often in African-American, Hispanics, Native Americans and Asians. Men can get lupus too. Lupus is not infectious or cancerous. People with lupus may have many different symptoms affecting various parts of the body. Some of the most common symptoms are extreme fatigue, painful or swollen joints (arthritis), unexplained fevers, skin rashes and kidney problems. Lupus is characterized by “flares” or periods of illness and remission. Warning signs of a flare can be increased fatigue, pain, rash, fever, abdominal discomfort, headache or dizziness. Learning how to recognize these signs can help people maintain better health and reduce or ward off a flare. Currently, there is no cure for lupus but it can be managed effectively with drugs, and most people with lupus lead an active, healthy life.
In its simplest definition, the CBC is used to measure red and white blood cell count, the total amount of hemoglobin in the blood, hematocrit (the amount of blood composed of red blood cells), and mean corpuscular volume (the size of red blood cells). The CBC can also count additional blood cell types like neutrophils, eosinophils, basophils, lymphocytes, monocytes, and platelets.
Sometimes changes in blood counts may contribute to symptoms of fatigue (low red blood cell count, anemia), serious infections (low white blood cell count), or easy bruising (low platelet count). However, many patients do not have symptoms that indicate blood abnormalities, so it is important for lupus patients to have periodic blood tests in order to detect any problems.
The panel concluded that both options (GCs plus CYC and GCs plus RTX) were associated with large benefits and moderate harms in comparison to GCs plus placebo in patients with acute neurological manifestations. No studies comparing these two options were identified. In terms of SLE and severe neurological manifestations, clinical trials with GCs plus CYC focused on both general neurologic manifestations, and on seizures, psychosis, myelitis, peripheral neuropathy, brain stem disease and optic neuritis, specifically. No data were found regarding other neuropsychiatric manifestations. The panel significantly weighted the fact that the certainty of the evidence was better for CYC than RTX and that RTX was only evaluated in refractory patients.
To unravel which people with positive ANA tests actually have lupus, additional blood work can be done. Doctors look for other potentially troublesome antibodies, so they will test for anti-double-stranded DNA and anti-Smith antibodies. These tests are less likely to be positive unless a patient truly has lupus. However, a person who has negative test results could still have lupus, even though this is not so in the case of ANA tests.
Over half of the people with SLE develop a characteristic red, flat facial rash over the bridge of their nose. Because of its shape, it is frequently referred to as the "butterfly rash" of SLE. The rash is painless and does not itch. The facial rash, along with inflammation in other organs, can be precipitated or worsened by exposure to sunlight, a condition called photosensitivity. This photosensitivity can be accompanied by worsening of inflammation throughout the body, called a "flare" of the disease.
Saturated fats, on the other hand, can raise cholesterol levels and may contribute to inflammation. So they should be limited. Sources of saturated fats include fried foods, commercial baked goods, creamed soups and sauces, red meat, animal fat, processed meat products, and high-fat dairy foods. That includes whole milk, half and half, cheeses, butter, and ice cream.
Belimumab, a type of agent referred to as a B-lymphocyte stimulator (BLyS) protein inhibitor, was approved by the U.S. Food and Drug Administration (FDA) in March 2011 for patients with lupus who are receiving other standard therapies, including those listed above. Given by IV infusion, belimumab may reduce the number of abnormal B cells thought to be a problem in lupus.
Heart and Lungs. Heart and lung involvement often is caused by inflammation of the covering of the heart (pericardium) and lungs (pleura). When these structures become inflamed, patients may develop chest pain, irregular heartbeat, and accumulation of fluid around the lungs (pleuritis or pleurisy) and heart (pericarditis). The heart valves and the lung itself can also be affected by lupus, resulting in shortness of breath.
The panel suggests SOC alone over adding other immunosuppressant (IS) in adult patients with SLE with MSK manifestations (weak recommendation based on low certainty of the evidence). It suggests also adding either MTX, LFN, belimumab or ABT to those failing to respond to SOC (weak recommendation based on low to moderate certainty of the evidence). Cost and availability may favour MTX (table 1).
What is known is that lupus is a chronic autoimmune disease (Healthdirect, 2016); meaning, that for people with lupus, their immune system attacks their healthy cells and tissues and not just foreign bodies/invaders (NIH, 2014). Evidently, this can lead to bodily damage. In the most common form of lupus, SLE (systemic lupus erythematosus), nearly all parts of the body can be affected (Healthdirect, 2016).
In addition to the 11 criteria, other tests can be helpful in evaluating people with SLE to determine the severity of organ involvement. These include routine testing of the blood to detect inflammation (for example, the erythrocyte sedimentation rate, or ESR, and the C-reactive protein, or CRP), blood-chemistry testing, direct analysis of internal body fluids, and tissue biopsies. Abnormalities in body fluids (joint or cerebrospinal fluid) and tissue samples (kidney biopsy, skin biopsy, and nerve biopsy) can further support the diagnosis of SLE. The appropriate testing procedures are selected for the patient individually by the doctor.
SLE is undoubtedly a potentially serious illness with involvement of numerous organ systems. However, it is important to recognize that most people with SLE lead full, active, and healthy lives. Periodic increases in disease activity (flares) can usually be managed by varying medications. Since ultraviolet light can precipitate and worsen flares, people with systemic lupus should avoid sun exposure. Sunscreens and clothing covering the extremities can be helpful. Abruptly stopping medications, especially corticosteroids, can also cause flares and should be avoided. People with SLE are at increased risk of infections as SLE-related complications, especially if they are taking corticosteroids or immunosuppressive medications. Therefore, any unexpected fever should be reported to medical professionals and evaluated.
In lupus as the attack goes on, all the branches of the immune system join the fight. This leads to significant and intense inflammation. The cause of Lupus is unknown, as well as what drives its diverse presentation. We know that multiple factors are required, including: the “right” genetic makeup, environmental exposures, and organ specific characteristics. People with lupus may also have an impaired process for clearing old and damaged cells from the body, which in turn provides continuous stimuli to the immune system and leads to abnormal immune response.
A biopsy is a procedure that removes a small piece of living tissue from your body. The tissue is examined with a microscope for signs of damage or disease. Biopsies can be done on all parts of the body. A biopsy is the only test that can tell for sure if a suspicious area is cancer. But biopsies are performed for many other reasons too. There are different ways to do a biopsy. A needle biopsy removes tissue with a needle passed through your skin to the site of the problem. Other kinds of biopsies require surgery.
Symptoms vary but can include fatigue, joint pain, a red rash on the face (also called the "butterfly rash") and fever. These symptoms can periodically get worse (flare-up) and then improve. Lupus flares can range from mild to severe, often resulting in periods in which the disease is relatively quiescent. Currently, no cures exist for lupus, and treatment often involves corticosteroids, other immunosuppressants or organ transplants. But research is providing hope for better diagnosis, treatments and even cures.
Angiogenesis is the growth of blood vessels from the existing vasculature. It occurs throughout life in both health and disease, beginning in utero and continuing on through old age. No metabolically active tissue in the body is more than a few hundred micrometers from a blood capillary, which is formed by the process of angiogenesis. Capillaries are needed in all tissues for diffusion exchange of nutrients and metabolites. Changes in metabolic activity lead to proportional changes in angiogenesis and, hence, proportional changes in capillarity. Oxygen plays a pivotal role in this regulation. Hemodynamic factors are critical for survival of vascular networks and for structural adaptations of vessel walls.
While there is no specific lupus diet, scientists have found that low-dose diet supplementation with omega-3 fish oils could help patients with lupus by decreasing inflammation and disease activity and possibly decreasing heart-disease risk. It is generally recommended that patients with lupus eat a balanced diet that includes plant-based foods and lean sources of protein.
Discoid Lupus is the most common form of Cutaneous Lupus. People living with Discoid Lupus complain of a red, raised and scaly lesion on the face, scalp or parts of the body. Manifestations on the face form across the cheeks, nose and ears. Over time, these lesions can produce scarring and skin discoloration (darkly colored and/or lightly colored areas). Typically, these lesions occur on areas of the body that are exposed to sunlight or fluorescent lights. If lesions appear in the scalp or involve the hair follicles, areas of hair loss may develop which could be permanent if the hair follicle is completely destroyed. They are often not itchy or painful.
An antinuclear antibody (ANA) test is a sensitive screening tool used to detect autoimmune diseases, including lupus. Antinuclear antibodies (ANAs) are antibodies that are directed against certain structures within a cell's nucleus (thus, antinuclear antibody). ANAs are found in particular patterns in people with autoimmune diseases (those in which a person's immune system works against his or her own body).
Steroids Synthetic cortisone medications are some of the most effective treatments for reducing the swelling, warmth, pain, and tenderness associated with the inflammation of lupus. Cortisone usually works quickly to relieve these symptoms. However, cortisone can also cause many unwelcome side effects, so it is usually prescribed only when other medications—specifically NSAIDs and anti-malarials—are not sufficient enough to control lupus.
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