To help doctors diagnose lupus, this list of 11 common criteria, or measures, was developed by the American College of Rheumatology (ACR). ACR is a professional association of rheumatologists. Rheumatologists are the doctors who specialize in treating diseases of the joints and muscles, like lupus. If you have at least four of the criteria on the list, either at the present time or at some time in the past, there is a strong chance that you have lupus.
Fatigue is different from drowsiness. Drowsiness is feeling the need to sleep. Fatigue is a lack of energy and motivation. Drowsiness and apathy (a feeling of not caring about what happens) can be symptoms that go along with fatigue. Fatigue can be a normal and important response to physical activity, emotional stress, boredom, or lack of sleep. Fatigue is a common symptom, and it is usually not due to a serious disease. But it can be a sign of a more serious mental or physical condition. When fatigue is not relieved by enough sleep, good nutrition, or a low-stress environment, it should be evaluated by your doctor.
B cells are essential for the development and pathogenesis of both systemic and organ-specific autoimmune diseases. Autoreactive B cells are typically thought of as sources of autoantibody, but their most important pathogenetic roles may be to present autoantigens to T cells and to secrete proinflammatory cytokines. A rate-limiting step in the genesis of autoimmunity then is the activation of autoreactive B cells. Here, mechanisms are discussed that normally prevent such activation and how they break down during disease. Integrating classic work with recent insights, emphasis is placed on efforts to pinpoint the precursor cells for autoantibody-secreting cells and the unique stimuli and pathways by which they are activated.
In more severe cases, medications that modulate the immune system (primarily corticosteroids and immunosuppressants) are used to control the disease and prevent recurrence of symptoms (known as flares). Depending on the dosage, people who require steroids may develop Cushing's syndrome, symptoms of which may include obesity, puffy round face, diabetes mellitus, increased appetite, difficulty sleeping and osteoporosis. These may subside if and when the large initial dosage is reduced, but long-term use of even low doses can cause elevated blood pressure and cataracts.
Aggrecan is a type of protein known as a proteoglycan, which means it has several sugar molecules attached to it. It is the most abundant proteoglycan in cartilage, a tough, flexible tissue that makes up much of the skeleton during early development. Most cartilage is later converted to bone (a process called ossification), except for the cartilage that continues to cover and protect the ends of bones and is present in the nose, airways, and external ears. Aggrecan attaches to the other components of cartilage, organizing the network of molecules that gives cartilage its strength. These interactions occur at a specific region of the aggrecan protein called the C-type lectin domain (CLD). Because of the attached sugars, aggrecan attracts water molecules and gives cartilage its gel-like structure. This feature enables the cartilage to resist compression, protecting bones and joints. Although its role is unclear, aggrecan affects bone development.
A one-celled organism without a true nucleus or cell organelles, belonging to the kingdom Procaryotae (Monera). The cytoplasm is surrounded by a rigid cell wall composed of carbohydrates and other chemicals that provide the basis for the Gram stain. Some bacteria produce a polysaccharide or polypeptide capsule, which inhibits phagocytosis by white blood cells. Bacteria synthesize DNA, RNA, and proteins, and they can reproduce independently but may need a host to provide food and a favorable environment. Millions of nonpathogenic bacteria live on human skin and mucous membranes; these are called normal flora. Bacteria that cause disease are called pathogens.
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.  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]
This screening test is used to detect substances or cellular material in the urine associated with metabolic and kidney disorders. It's a routine test, and doctors utilize it to detect abnormalities that often appear before patients suspect a problem. For those with acute or chronic conditions, regular urinalysis can help monitor organ function, status, and response to treatment. A higher number of red blood cells or a higher protein level in your urine may indicate that lupus has affected your kidneys.
“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.
Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.
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.
Although guidelines for SLE treatment do exist and there is scarce evidence to support specific therapies for Latin American patients with lupus,16–21 this regional effort has considered the impact of racial/ethnic background1 10 22–28 and SES3 9 on lupus outcomes and treatment response.25 26 Other medication variables such as cost and availability were also taken into account since they affect adherence and are relevant in decision-making.27 28 GLADEL and the Pan-American League of Associations of Rheumatology have joined efforts to produce these guidelines,29 which are presented by organ systems, although manifestations usually occur in more than one. Nevertheless, treatment is usually tailored to the more severe manifestation(s), which usually benefits the less severe.
Medications that suppress immunity (immunosuppressive medications) are also called cytotoxic drugs. They are sometimes referred to as chemotherapy because they are also used to treat cancer, generally in much higher doses than those used to treat lupus. Immunosuppressive medications are used for treating people with more severe manifestations of SLE, such as damage to internal organ(s). Examples of immunosuppressive medications include azathioprine (Imuran), cyclophosphamide (Cytoxan), chlorambucil (Leukeran), cyclosporine (Sandimmune), and the disease-modifying drug methotrexate (Rheumatrex, Trexall). All immunosuppressive medications can seriously depress blood-cell counts and increase risks of infection and bleeding. Immunosuppressive medications may not be taken during pregnancy or conceptionbecause of risk to the fetus. Other side effects are specific for each drug. For examples, methotrexate can cause liver toxicity, while cyclosporine can impair kidney function.
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.
Chronic diseases are noncommunicable illnesses that are prolonged in duration, do not resolve spontaneously, and are rarely cured completely. Although chronic diseases are more common among older adults, they affect people of all ages and are now recognized as a leading health concern of the nation. Growing evidence indicates that a comprehensive approach to prevention can save tremendous costs and needless suffering.
Immunosuppressive Medications Immunosuppressives are medications that help suppress the immune system. Many were originally used in patients who received organ transplants to help prevent their bodies from rejecting the transplanted organ. However, these drugs are now also used for the treatment of certain autoimmune diseases, such as lupus and rheumatoid arthritis.
Rheumatologists have long been concerned that the female hormone estrogen or treatment with estrogen may cause or worsen lupus. Recent research showed that estrogen therapy can trigger some mild or moderate flares of lupus, but does not cause symptoms to get much worse. Yet, estrogen can raise the risk of blood clots. Thus, you should not take estrogen if your blood tests show antiphospholipid antibodies (meaning you already have a high risk of blood clots).
“There’s no specific diet for lupus, but the Mediterranean-style diet comes close to what’s most ideal," says Sotiria Everett, RD, a clinical assistant professor in the department of family, population, and preventive medicine at Stony Brook School of Medicine in New York. "You want to eat a diet that’s low in fat and sugar and has lots of fruits and vegetables. You should get some of your protein from fish and eat lots of beans and legumes because they’re high in fiber, vitamin B, and iron."
Any of a group of immunoglobulin autoantibodies that react with phospholipids, which are one of the primary components of the cell membrane (the other components are glycolipids and steroids). These antibodies are found in patients with a variety of connective tissue and infectious disorders, including systemic lupus erythematosus, the antiphospholipid antibody syndrome, syphilis, and malaria. They cause abnormal blood clotting, thrombocytopenia; and in women of childbearing age, repeated miscarriages. The anticardiolipin antibodies are one type of antiphospholipid antibody.
Useful medication for the disease was first found in 1894, when quinine was first reported as an effective therapy. Four years later, the use of salicylates in conjunction with quinine was noted to be of still greater benefit. This was the best available treatment until the middle of the twentieth century, when Hench discovered the efficacy of corticosteroids in the treatment of SLE.
For some patients whose kidneys or central nervous systems are affected by lupus, a type of drug called an immunosuppressive may be used. Immunosuppressives, such as cyclophosphamide and mycophenolate mofetil, restrain the overactive immune system by blocking the production of immune cells. These drugs may be given by mouth or by IV infusion. The risk for side effects increases with the length of treatment.
Subacute Cutaneous Lupus can cause skin lesions on any part of the body. These lesions often form red, ring-shaped, scaly patches on the skin. These lesions do not itch and often appear on the chest as well as the upper back and neck; however, they may also be seen on the face and arms. Typically, these lesions occur on areas of the body that are exposed to sunlight or fluorescent lights. Furthermore, it is not uncommon for patients with SCLE to have associated joint disease.
Research is indicating benefits of rituximab (Rituxan) in treating lupus. Rituximab is an intravenously infused antibody that suppresses a particular white blood cell, the B cell, by decreasing their number in the circulation. B cells have been found to play a central role in lupus activity, and when they are suppressed, the disease tends toward remission. This may particularly helpful for people with kidney disease.
Drugs used to treat lupus include nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen, alone or combined with other drugs for pain, swelling, and fever. Drugs that work inside cells, including antimalarial drugs such as hydroxychloroquine (Plaquenil) are used for fatigue, joint pain, skin rashes, and inflammation of the lungs. Continuous treatment with antimalarials may prevent lupus flare up from recurring.
Certain foods, including garlic and alfalfa sprouts, should be avoided by people with lupus. [For a more complete list of items to be avoided, please see the article “Things to Avoided” in the Lupus 101 section.] Recently controversy has also arisen over whether aspartame induces lupus. However, scientists have concluded that there is no evidence to suggest that aspartame causes lupus.
People with lupus should know that most rashes, and sometimes other symptoms, are aggravated by sun exposure, so you’ll want to avoid it or use sun protection. It’s critical to talk to your doctor about skin rashes and lesions that you observe, as many are treated differently, and some can be signs that the disease is progressing or changing. You may need other treatments, too.
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.
Women with lupus have a higher risk of miscarriage and preterm labor, says Kaplan. Pregnant women with lupus also have a higher risk of preeclampsia, or high blood pressure, and signs that the kidneys and liver may not be functioning well. (20) If you have lupus and do get pregnant (or if you have lupus and are trying to get pregnant), see a high-risk maternal-fetal medicine specialist who has expertise in how to best handle such pregnancies.
Pain is typically treated with opioids, varying in potency based on the severity of symptoms. When opioids are used for prolonged periods, drug tolerance, chemical dependency, and addiction may occur. Opiate addiction is not typically a concern since the condition is not likely to ever completely disappear. Thus, lifelong treatment with opioids is fairly common for chronic pain symptoms, accompanied by periodic titration that is typical of any long-term opioid regimen.
Erythrocyte sedimentation rate. This blood test determines the rate at which red blood cells settle to the bottom of a tube in an hour. A faster than normal rate may indicate a systemic disease, such as lupus. The sedimentation rate isn't specific for any one disease. It may be elevated if you have lupus, an infection, another inflammatory condition or cancer.
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