Sjogren’s syndrome is a disease that causes dryness in your mouth and eyes. It can also lead to dryness in other places that need moisture, such as your nose, throat and skin. Most people who get Sjogren’s syndrome are older than 40. Nine of 10 are women. Sjogren’s syndrome is sometimes linked to rheumatic problems such as rheumatoid arthritis. In Sjogren’s syndrome, your immune system attacks the glands that make tears and saliva. It may also affect your joints, lungs, kidneys, blood vessels, digestive organs and nerves. The main symptoms are:
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.
Only one population-based screening study13 of systemic lupus erythematosus was identified. This study reported a prevalence of 200 cases per 100,000 women (18 to 65 years of age) in England. One review14 estimated the overall U.S. prevalence of definite systemic lupus erythematosus plus incomplete systemic lupus erythematosus (disease meeting only some diagnostic requirements for systemic lupus erythematosus) to be 40 to 50 cases per 100,000 persons.
Painless passage of blood or protein in the urine may often be the only presenting sign of kidney involvement. Acute or chronic renal impairment may develop with lupus nephritis, leading to acute or end-stage kidney failure. Because of early recognition and management of SLE, end-stage renal failure occurs in less than 5% of cases; except in the black population, where the risk is many times higher.
Cardiac tamponade is pressure on the heart that occurs when blood or fluid builds up in the space between the heart muscle (myocardium) and the outer covering sac of the heart (pericardium). This prevents the heart ventricles from expanding fully. The excess pressure from the fluid prevents the heart from working properly. As a result, the body does not get enough blood.
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.
“It’s always difficult for children and parents to live with the idea that lupus is chronic,” says Pascual. That means the child has many more years worth of living with the condition than if he or she were diagnosed later in life. And because this disease is lifelong and may involve complications such as nephritis, doctors need to manage it aggressively.
At least half of people with lupus experience fatigue. (4) Fatigue may be brought on by the disease itself or from associated depression, anxiety, lack of exercise, and problems with sleep. ( 5) Because people with lupus need to avoid sun exposure, they may have low levels of vitamin D, which can contribute to fatigue. Lupus treatments may also play a role.
The goal of the informed consent process is to protect participants. It begins when a potential participant first asks for information about a study and continues throughout the study until the study ends. The researcher and potential participant have discussions that include answering the participant’s questions about the research. All the important information about the study must also be given to the potential participant in a written document that is clear and easy to understand. This informed consent document is reviewed and approved by the human subjects review board for a study before it is given to potential participants. Generally, a person must sign an informed consent document to enroll in a study.
While no single test can determine whether a person has lupus, several laboratory tests may help the doctor confirm a diagnosis, or at least rule out other ailments. The most useful tests identify certain autoantibodies that are often present in the blood of lupus patients. A biopsy of the skin or kidneys may also be ordered if those organs are affected. The doctor will look at the entire picture – medical history, symptoms, and test results – to determine if you have lupus. Other laboratory tests are used to monitor the progress of the disease once it has been diagnosed.
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.
Lupus affects people in many different ways, so there is not one diet which is guaranteed to work for everyone, but the Mediterranean diet (plenty of fruit and vegetables, grains, nuts and seeds, two portions of fish per week and small amounts of meat and dairy produce) is probably the simplest one to follow and is suitable for all the family as it is a pattern of healthy eating.
Autoreactive B cells can accidentally emerge during somatic hypermutation and migrate into the germinal center light zone. Autoreactive B cells, maturated coincidentally, normally do not receive survival signals by antigen planted on follicular dendritic cells and perish by apoptosis. In the case of clearance deficiency, apoptotic nuclear debris accumulates in the light zone of GC and gets attached to FDC. This serves as a germinal centre survival signal for autoreactive B-cells. After migration into the mantle zone, autoreactive B cells require further survival signals from autoreactive helper T cells, which promote the maturation of autoantibody-producing plasma cells and B memory cells. In the presence of autoreactive T cells, a chronic autoimmune disease may be the consequence.
We conducted a systematic evidence-based review of the published literature on systemic lupus erythematosus. After searching several evidence-based databases (Table 1), we reviewed the MEDLINE database using the PubMed search engine. Search terms included “lupus not discoid not review not case” and “lupus and treatment and mortality,” with the following limits: 1996 to present, abstract available, human, and English language. One author reviewed qualifying studies for relevance and method.
The most commonly sought medical attention is for joint pain, with the small joints of the hand and wrist usually affected, although all joints are at risk. More than 90 percent of those affected will experience joint or muscle pain at some time during the course of their illness. Unlike rheumatoid arthritis, lupus arthritis is less disabling and usually does not cause severe destruction of the joints. Fewer than ten percent of people with lupus arthritis will develop deformities of the hands and feet. People with SLE are at particular risk of developing osteoarticular tuberculosis.
Arthritis is inflammation of one or more joints and it involves the breakdown of cartilage. Cartilage normally protects a joint, allowing it to move smoothly. Cartilage also absorbs shock when pressure is placed on the joint, such as when you walk. Without the normal amount of cartilage, the bones rub together, causing pain, swelling (inflammation), and stiffness. Usually the joint inflammation goes away after the cause goes away or is treated. Sometimes it does not. When this happens, you have chronic arthritis. Arthritis may occur in men or women. Osteoarthritis is the most common type.
Neuropsychiatric syndromes can result when SLE affects the central or peripheral nervous system. The American College of Rheumatology defines 19 neuropsychiatric syndromes in systemic lupus erythematosus. The diagnosis of neuropsychiatric syndromes concurrent with SLE (now termed as NPSLE), is one of the most difficult challenges in medicine, because it can involve so many different patterns of symptoms, some of which may be mistaken for signs of infectious disease or stroke.
Libman-Sacks endocarditis is the most characteristic cardiac manifestation of lupus. It is characterized by clusters of verrucae on the ventricular surface of the mitral valve. These lesions consist of accumulation of immune complexes, platelets, and mononuclear cells. This can lead to heart failure, valvular dysfunction, emboli, and secondary infective endocarditis. Diagnosis is best made via echocardiography, which may reveal the characteristic valvular masses (arrows). IVS = interventricular septum; LA = left atrium; LV = left ventricle.
The medical doctors who treat lupus are rheumatologists who specialize in arthritis and other inflammatory disorders. However, depending on the individual, case treatment may involve a wide range of health professionals including clinical immunologists (doctors specializing in immune system disorders), nurses, psychologists, social workers, nephrologists (kidney disease specialists), hematologists (specialists in blood disorders), dermatologists, and neurologists.
People with SLE need more rest during periods of active disease. Researchers have reported that poor sleep quality was a significant factor in developing fatigue in people with SLE. These reports emphasize the importance for people and physicians to address sleep quality and the effect of underlying depression, lack of exercise, and self-care coping strategies on overall health. During these periods, carefully prescribed exercise is still important to maintain muscle tone and range of motion in the joints.
If cyclophosphamide doesn’t work for patients with lupus nephritis, they now have the option of trying another drug, and more options are being tested in clinical trials, says Dr. Caricchio. For example, doctors may use CellCept (mycophenolate mofetil), Imuran (azathioprine), or Restasis (cyclosporine). Prograf (tacrolimus) may also be an effective option for lupus nephritis, according to study data appearing in the January 2016 edition of Autoimmunity Reviews. (5)
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