Disease that results when the immune system mistakenly attacks the body’s own tissues. Examples include multiple sclerosis, type 1 diabetes, rheumatoid arthritis, and systemic lupus erythematosus. Autoimmune diseases can affect almost any part of the body, including the heart, brain, nerves, muscles, skin, eyes, joints, lungs, kidneys, glands, the digestive tract, and blood vessels. The classic sign of an autoimmune disease is inflammation, which can cause redness, heat, pain, and swelling.
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.
Limitations of the test: Like CRP, the ESR is not specific to lupus. Because there are many causes for a positive result, including infection, the test is not diagnostic for lupus. Nor can it distinguish a lupus flare from an infection. Also, the level doesn't directly correlate with lupus disease activity. So it isn't necessarily useful for monitoring disease activity.
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Most autoimmune diseases affect one specific system. For example, Rheumatoid Arthritis involves the joints, and Multiple Sclerosis affects the brain and spinal cord. Lupus, on the other hand, affects more than one system simultaneously. No matter what organ or system is being attacked, all autoimmune diseases are similar in that they are an immune response caused by systemic inflammation that leads your body to attack itself.
While the onset and persistence of SLE can show disparities between genders, socioeconomic status also plays a major role. Women with SLE and of lower socioeconomic status have been shown to have higher depression scores, higher body mass index, and more restricted access to medical care than women of higher socioeconomic statuses with the illness. People with SLE had more self-reported anxiety and depression scores if they were from a lower socioeconomic status.
The panel recommends SOC (GCs and antimalarials (AM)) in addition to an IS (CYC in high or low doses, MMF or TAC) over GCs alone, for induction in patients with SLE-related kidney disease (strong recommendation based on moderate certainty of the evidence). Although more African-American descendants and Hispanic patients responded to MMF than CYC (25), limited access to MMF and TAC in several Latin American countries, due primarily to cost issues, makes CYC the best alternative for induction (high or low dose) in these regions (table 2).
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.
(1) SOC; (2) SOC plus methotrexate (MTX); (3) SOC plus leflunomide (LFN); (4) SOC plus belimumab; (5) SOC plus abatacept (ABT); (6) other options: azathioprine (AZA), mycophenolate mofetil (MMF), cyclosporine A (CsA) or rituximab (RTX) (online supplementary tables S2.1.1, S2.1.4, S2.1.6, S2.1.7, S2.2.11, S2.1.11, S2.1.12, S2.1.14, S2.1.15, S2.1.17, S2.2.1, S2.2.2, S2.2.4, S3.1.1, S3.1.3–S3.1.6, S3.2.1, S3.2.2, S12.2–S12.5, S12.8–S12.10).
The mission of the National Institute of Arthritis and Musculoskeletal and Skin Diseases is to support research into the causes, treatment, and prevention of arthritis and musculoskeletal and skin diseases; the training of basic and clinical scientists to carry out this research; and the dissemination of information on research progress in these diseases.
The immune response is how your body recognizes and defends itself against bacteria, viruses, and substances that appear foreign and harmful.The immune system protects the body from possibly harmful substances by recognizing and responding to antigens. Antigens are substances (usually proteins) on the surface of cells, viruses, fungi, or bacteria. Nonliving substances such as toxins, chemicals, drugs, and foreign particles (such as a splinter) can also be antigens. The immune system recognizes and destroys substances that contain antigens. Your own body’s cells have proteins that are antigens. These include a group of antigens called HLA antigens. Your immune system learns to see these antigens as normal and usually does not react against them.
Combination treatment: Health care providers may combine a few medications to control lupus and prevent tissue damage. Each treatment has risks and benefits. Most immune-suppressing medications may cause side effects and require close monitoring. Side effects of these drugs may include a raised risk of infections as well as nausea, vomiting, hair loss, diarrhea, high blood pressure, and osteoporosis (weak bones). Rheumatologists may lower the dose of a drug or stop a medicine because of side effects or when the disease goes into remission. As a result, it is important to receive careful and frequent health exams and lab tests to track your symptoms and change your treatment as needed.
Lupus is chronic, complex, and difficult to diagnose. No single lab test can tell if you have lupus. Many lupus symptoms imitate symptoms of other diseases and often come and go. Your primary care doctor or rheumatologist will use your medical history, a physical exam, and many routine as well as special tests to rule out other diseases. Many physicians also use the American College of Rheumatology's "Eleven Criteria of Lupus" to aid in the diagnosis of lupus. The criteria include symptoms as well as specific laboratory findings that provide information about the functioning of a person's immune system. In most cases, the diagnosis of lupus is made when four or more of the criteria have occurred at some time.
In healthy conditions, apoptotic lymphocytes are removed in germinal centers (GC) by specialized phagocytes, the tingible body macrophages (TBM), which is why no free apoptotic and potential autoantigenic material can be seen. In some people with SLE, accumulation of apoptotic debris can be observed in GC because of an ineffective clearance of apoptotic cells. In close proximity to TBM, follicular dendritic cells (FDC) are localised in GC, which attach antigen material to their surface and, in contrast to bone marrow-derived DC, neither take it up nor present it via MHC molecules.
Research and documentation of the disease continued in the neoclassical period with the work of Ferdinand von Hebra and his son-in-law, Moritz Kaposi. They documented the physical effects of lupus as well as some insights into the possibility that the disease caused internal trauma. Von Hebra observed that lupus symptoms could last many years and that the disease could go "dormant" after years of aggressive activity and then re-appear with symptoms following the same general pattern. These observations led Hebra to term lupus a chronic disease in 1872.
SLE may cause pericarditis—inflammation of the outer lining surrounding the heart, myocarditis—inflammation of the heart muscle, or endocarditis—inflammation of the inner lining of the heart. The endocarditis of SLE is non-infectious, and is also called (Libman–Sacks endocarditis). It involves either the mitral valve or the tricuspid valve. Atherosclerosis also occurs more often and advances more rapidly than in the general population.
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.
In the absence of systemic lupus erythematosus, the most common reason for a positive ANA test is the presence of another connective tissue disease. Diseases that often are associated with a positive ANA test include Sjögren's syndrome (68 percent of affected patients), scleroderma (40 to 75 percent), rheumatoid arthritis (25 to 50 percent), and juvenile rheumatoid arthritis (16 percent).20 An ANA test also can be positive in patients with fibromyalgia. In patients with diseases other than systemic lupus erythematosus, ANA titers usually are lower, and the immunofluorescent pattern is different.20
Lupus in children tends to be more aggressive than in adults, says Dr. Pascual. The exact reasons for this are not understood. One theory is that people are born with genetic susceptibility to the disease that may be triggered by environmental factors such as a virus. “Children with the condition may have inherited a more complex set of predisposing genes,” she says. But this theory has yet to be proved.
Corticosteroids may also be used to get rid of lupus flares, or the appearance of symptoms after a period of remission, says Francis Luk, MD, an assistant professor of rheumatology and immunology at Wake Forest Baptist Medical Center in Winston-Salem, North Carolina. “Depending on severity and type of flare and how many flares the patient has recently experienced, rheumatologists may adjust medications,” he adds.
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