As required by Section 801 of the Food and Drug Administration Amendments Act, in general, a description of any agreement between the sponsor of a clinical study and the principal investigator (PI) that does not allow the PI to discuss the results of the study or to publish the study results in a scientific or academic journal after the trial is completed. (This does not apply if the PI is an employee of the sponsor.)
However, the mainstays of treatment are corticosteroids such as prednisone (Deltasone), hydrocortisone, methylprednisolone (Medrol), and dexamethasone (Decadron, Hexadrol). These drugs heavily suppress inflammation but can cause short-term side effects including swelling, increased appetite, and weight gain and long-term side effects including stretch marks on the skin, weakened or damaged bones, high blood pressure, damage to the arteries, diabetes, infections, and cataracts.
Most people who have SLE have low levels of vitamin D and should take a vitamin D supplement regularly. Vitamin D is essential for proper function of the immune system and several studies have shown that people who have more severe lupus tend to have lower levels of vitamin D compared to those who have milder disease. It is advised to talk with your consultant or GP about your vitamin D levels as you may already be prescribed calcium supplements which may contain vitamin D. Some dietary sources of vitamin D can be found HERE. It is important to bear in mind that most vitamin D is usually synthesised from sunlight on the skin, but with lupus you should be protecting yourself from exposure to UV.
With variants known as discoid lupus, subacute cutaneous lupus, and systemic lupus erythematosus, lupus is one of several disorders of the immune system considered “autoimmune” in nature. These diseases occur when the immune system malfunctions and turns its infection-defense capabilities against the body, producing antibodies against healthy cells and tissues. These antibodies promote chronic inflammation and can damage organs and tissues. In lupus, these antibodies are known as antinuclear antibodies (ANA) because they target parts of the cell’s nucleus. Experts don’t yet fully understand all of the factors and triggers that cause inflammation and tissue damage in lupus, and research is ongoing.
It also is known that some women with systemic lupus erythematosus can experience worsening of their symptoms prior to their menstrual periods. This phenomenon, together with the female predominance of systemic lupus erythematosus, suggests that female hormones play an important role in the expression of SLE. This hormonal relationship is an active area of ongoing study by scientists.
The symptoms involved in CREST syndrome are associated with the generalized form of the disease Systemic sclerosis (scleroderma). CREST is an acronym for the clinical features that are seen in a patient with this disease. The “C” stands for calcinosis, where calcium deposits form under the skin on the fingers or other areas of the body. The “R”, stands for Raynaud’s phenomenon, spasm of blood vessels in the fingers or toes in response to cold or stress. The “E” represents esophageal dysmotility, which can cause difficulty in swallowing. The “S” is for sclerodactyly, tightening of the skin causing the fingers to bend. Finally, the letter “T” is for telangiectasia, dilated vessels on the skin of the fingers, face, or inside of the mouth.
Neonatal lupus Technically neonatal lupus is not a form of lupus. The condition is the result of autoantibodies passing from a pregnant woman with lupus (or related condition) through the placenta and to the baby developing in the womb, causing mostly temporary symptoms, explains Virginia Pascual, MD, the director of the Gale and Ira Drukier Institute for Children’s Health at Weill Cornell Medicine in New York City. Some infants are born with symptoms, such as skin rash, liver problems, or white blood cell counts. But those symptoms disappear within a few months and leave no lasting effects.
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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 prognosis for those with lupus often depends on the amount of organ involvement. In other words, is the disease targeting organs rather than skin and joints? Survival for lupus patients with central nervous system symptoms, major organ involvement, and/or kidney disease, is likely to be shorter than those with only skin and/or joint disease related to lupus. The most common cause of death associated with lupus is an infection due to immunosuppression, caused by medications used to manage the disease, especially early in the disease.
In patients with SLE, the risk of developing cardiovascular disease (CVD) is at least twice that in the general population, and over half of patients have 3 or more CVD risk factors.3,4 “Following a heart-healthy diet that is rich in fruits, vegetables, whole grains, lean protein, and fatty fish and limiting saturated and trans fats can actually help reduce the risk of heart disease,” Gibofsky told Rheumatology Advisor.
Neutrophils, 55% to 70% of all leukocytes, are the most numerous phagocytic cells and are a primary effector cell in inflammation. Eosinophils, 1% to 3% of total leukocytes, destroy parasites and are involved in allergic reactions. Basophils, less than 1% of all leukocytes, contain granules of histamine and heparin and are part of the inflammatory response to injury. Monocytes, 3% to 8% of all leukocytes, become macrophages and phagocytize pathogens and damaged cells, esp. in the tissue fluid. Lymphocytes, 20% to 35% of all leukocytes, have several functions: recognizing foreign antigens, producing antibodies, suppressing the immune response to prevent excess tissue damage, and becoming memory cells.
Any of a group of glycoproteins with antiviral activity. The antiviral type I interferons (alpha and beta interferons) are produced by leukocytes and fibroblasts in response to invasion by a pathogen, particularly a virus. These interferons enable invaded cells to produce class I major histocompatibility complex surface antigens, increasing their ability to be recognized and killed by T lymphocytes. They also inhibit virus production within infected cells. Type I alpha interferon is used to treat condyloma acuminatum, chronic hepatitis B and C, and Kaposi’s sarcoma. Type I beta interferon is used to treat multiple sclerosis. Type II gamma interferon is distinctly different from and less antiviral than the other interferons. It is a lymphokine, excreted primarily by CD8+ T cells and the helper T subset of CD4+ cells that stimulates several types of antigen-presenting cells, particularly macrophages, to release class II MHC antigens that enhance CD4+ activity. It is used to treat chronic granulomatous disease.
For each of the subheadings listed below, the panel considered interventions based on experience, availability, affordability and a stepwise therapeutic approach of the different alternatives. Standard of care (SOC) was defined as the use of hydroxychloroquine (HCQ) and, if clinically indicated, low-dose glucocorticoids (GC) (prednisone ≤7.5 mg or equivalent for the shortest time).24 Chloroquine remains an alternative for some of the Latin American countries where HCQ is not available and careful monitoring of eye side effect is recommended. Overarching principles are shown in box 1. Tables summarising the evidence that was considered in the process are shown in online supplementary tables in https://doi.org/10.5061/dryad.bg8452h.
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.
Limitations of the test: Although almost all people with lupus have the antibody, a positive result doesn't necessarily indicate lupus. Positive results are often seen with some other diseases and in a smaller percentage of people without lupus or other autoimmune disorders. So a positive ANA by itself is not enough for a lupus diagnosis. Doctors must consider the result of this test along with other criteria.
Research has demonstrated evidence that a key enzyme's failure to dispose of dying cells may contribute the development of systemic lupus erythematosus. The enzyme, DNase1, normally eliminates what is called "garbage DNA" and other cellular debris by chopping them into tiny fragments for easier disposal. Researchers turned off the DNase1 gene in mice. The mice appeared healthy at birth, but after six to eight months, the majority of mice without DNase1 showed signs of systemic lupus erythematosus. Thus, a genetic mutation in a gene that could disrupt the body's cellular waste disposal may be involved in the initiation of systemic lupus erythematosus.
Disease-modifying antirheumatic drugs (DMARDs). DMARDs do more than just treat the symptoms of lupus. Research has shown that they can modify the course of the disease, prevent progression and slow joint damage. DMARDs are often used with NSAIDs. Hydroxychloriquine commonly is prescribed for people with lupus. It can cause vision changes in some people, so it is important to have regular vision examinations. Hydroxychloriquine is effective in preventing flares.
An intravenous pyelogram (IVP) is a special x-ray examination of the kidneys, bladder, and ureters (the tubes that carry urine from the kidneys to the bladder). An intravenous pyelogram is performed by injecting contrast material into a vein in the arm. A series of x-rays are taken at timed intervals as the contrast material goes through the kidneys, the ureters, and the bladder. The procedure helps to evaluate the condition of those organs.
Processed foods Think of these as any food that comes from a box or a can. Processed foods are higher in fat, sugar, and salt (check the nutritional information for amounts). Refined foods are on this list, too — typical white bread, pasta, and white rice. Goldman Foung says that “by replacing processed goods, packaged foods, and takeout food with meals full of fresh ingredients,” her diet is “tastier and healthier.”
Other medicines. You may need other medicines to treat illnesses or diseases that are linked to your lupus — such as high blood pressure or osteoporosis. Many people with lupus are also at risk for blood clots, which can cause a stroke or heart attack. Your doctor may prescribe anticoagulants (“blood thinners”), such as warfarin or heparin, to prevent your blood from clotting too easily. You cannot take warfarin during pregnancy.
If you have osteoporosis or osteopenia, your doctor will most likely recommend that you take calcium and vitamin D supplements in addition to your regular bone medications, since vitamin D helps your body to absorb calcium. It is important that you also try to eat foods rich in calcium, such as milk, light ice cream/frozen yogurt, cottage cheese, pudding, almonds, broccoli, fortified cereal, oranges, yogurt, hard cheese, soybeans and soymilk, navy beans, oysters, sardines, and spinach. These foods will help to keep your bones as healthy and strong as possible.
Since SLE patients can have a wide variety of symptoms and different combinations of organ involvement, no single test establishes the diagnosis of systemic lupus. To help doctors improve the accuracy of the diagnosis of SLE, 11 criteria were established by the American Rheumatism Association. These 11 criteria are closely related to the symptoms discussed above. Some people suspected of having SLE may never develop enough criteria for a definite diagnosis. Other people accumulate enough criteria only after months or years of observation. When a person has four or more of these criteria, the diagnosis of SLE is strongly suggested. Nevertheless, the diagnosis of SLE may be made in some settings in people with only a few of these classical criteria, and treatment may sometimes be instituted at this stage. Of these people with minimal criteria, some may later develop other criteria, but many never do.
Another targeted treatment, anifrolumab, is being investigated in clinical trials and appears to be promising, says Stacy Ardoin, MD, a rheumatologist at the Ohio State University Wexner Medical Center in Columbus, citing a study in the February 2017 issue of Arthritis & Rheumatology (7). “I don’t think it will work for everyone, but it’s good to have another treatment option.”
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