The immune system is a network of cells and tissues that protect the body against foreign organisms, such as bacteria and viruses. One mechanism used by the immune system is the release of antibodies. Antibodies are molecules that attack and destroy foreign organisms. For each type of organism, the immune system produces a special kind of antibody.
Lupus is an autoimmune disease, which means that the body's natural defense system (immune system) attacks its own tissues instead of attacking foreign substances like bacteria and viruses. This causes inflammation. Inflammation causes swelling, pain, and tissue damage throughout the body. If you develop severe lupus, you may have problems with your kidneys, heart, lungs, nervous system, or blood cells. Lupus is the common name for systemic lupus erythematosus, also called SLE.
Although some people with lupus have only mild symptoms, the disease is lifelong and can become severe. But most people can control their symptoms and prevent severe damage to their organs. They do this by seeing their doctors often for checkups, getting enough rest and exercise, and taking medicines.
The most common and most serious type of lupus are discoid or cutaneous lupus, drug-induced systemic lupus, neonatal lupus, and subacute cutaneous lupus.
Here is a brief look at each one.
Systemic Lupus: Systemic means “all over”. It can effect almost any organ or system of the body and causes serious blood abnormalities. This form can be life threatening if it involves the organs of the body.
Drug-Induced Lupus: Some people develop symptoms of Lupus after taking certain prescription drugs. It is known that 70 agents can cause symptoms but the top three are: Pronestyl for heart irregularities; Apresoline to treat high blood pressure; and Isoniazid used to treat tuberculosis.
Discoid Lupus: This form is mild and only effects the skin. A raised red rash may appear on the face, neck, ear, scalp or on other areas.
Neonatal Lupus: This is a non chronic form of Lupus limited to mothers who carry a certain autoantibody that crosses the placenta. Neonatal Lupus manifests as a rash which clears up after the first year of the baby's life.
Anyone can get lupus. But 9 out of 10 people who have it are women. African American women are three times more likely to get lupus than white women. It's also more common in Hispanic/Latino, Asian, and American Indian women.
Both African Americans and Hispanics/Latinos tend to develop lupus at a younger age and have more symptoms at diagnosis (including kidney problems).
They also tend to have more severe disease than whites. For example, African American patients have more seizures and strokes, while Hispanic/Latino patients have more heart problems. We don't understand why some people seem to have more problems with lupus than others.
Lupus is most common in women between the ages of 15 and 44. These are roughly the years when most women are able to have babies. Scientists think a woman's hormones may have something to do with getting lupus. But it's important to remember that men and older people can get it, too.
It's less common for children under age 15 to have lupus. One exception is babies born to women with lupus. These children may have heart, liver, or skin problems caused by lupus. With good care, most women with lupus can have a normal pregnancy and a healthy baby.
Common signs of lupus are:
Painful or swollen joints.
Chest pain with deep breathing.
Extreme fatigue (feeling tired all the time).
Unusual hair loss (mainly on the scalp).
Pale or purple fingers or toes from cold or stress.
Sensitivity to the sun.
Low blood count.
Depression, trouble thinking, and/or memory problems.
Red rash or color change on the face, often in the shape of a butterfly across the nose and cheeks.
Other signs are mouth sores, unexplained seizures (convulsions), "seeing things" (hallucinations), repeated miscarriages, and unexplained kidney problems.
Causes and Risk factors:
First of all, lupus isn't contagious. Second, even doctors and researchers are not sure what exactly causes lupus. What they do know is that it is an autoimmune disease in which a person's immune system malfunctions, and causes the body to attack its own tissues.
Genetics: Most researchers agree that genetics or heredity is at least one factor in determining a person propensity for developing lupus. To see if you fall in this category, review your family's medical history. Is there incidence of lupus in your immediate family? If so, you may be predisposed to lupus.
Remember, though, a family history of lupus does not mean an individual will get lupus, only that he or she is more susceptible. It is also important to understand that, presently, there is no scientific proof that genetics plays a key role, though researchers believe it is probable.
Environment: Another path researchers are walking down is environmental factors as a lupus trigger. Those environmental factors might include exposure to ultraviolet light (photosensitivity), smoking and exposure to smoking, stress, and exposure to toxins such as trichloroethylene in well water and silica dust. Certain hair products and topicals were once believed to be lupus triggers, but that is no longer the case.
Hormones and Illness: Research suggests that hormonal factors are linked to autoimmune disease, though research is still in its infancy and the link between the two is still nebulous. For example, some studies find a correlation between increased risk of lupus and high levels of estrogen, but another study, which focused on hormone replacement therapy, did not show a link. Still, abnormal estrogen metabolism is considered a risk factor.
So are viruses and bacteria. Those with viruses like cytomegalovirus, parvovirus and hepatitis C may eventually develop lupus, but a direct causal link has not been established. And the Epstein-Barr virus (EBV) in children is linked to childhood lupus. Most people become infected with EBV, which is often asymptomatic (without symptoms) but commonly causes infectious mononucleosis.
Medications: It has been established that some medications are suspected triggers of lupus and flares. In fact, a subset of the disease, drug-induced lupus is based on this notion. This type of lupus is usually brought on by long-term use of certain medications (covered more specifically in types of lupus).
The major difference between drug-induced lupus and standard lupus, or systemic lupus erythematosus (SLE) is that, once the patient stops taking those medications, symptoms decline within days and weeks. But there is no cure for SLE.
The Combo Platter: Finally, most doctors believe it is a combination of these factors that cause the onset of lupus. For example, it is assumed that a person with a family history of lupus that is exposed to certain medications or environmental factors is probably more susceptible to getting the disease than someone with only one of those causal factors.
But as we have said before, lupus is one of those mysterious diseases that doctors haven't quite pinned down. It won't be until the science catches up with the disease that we will truly understand what causes this autoimmune disease to occur.
Age: Although lupus affects people of all ages, including infants, children and older adults, it's most often diagnosed between the ages of 15 and 45.
Race: Lupus is more common in blacks and in Asians.
Sunlight: Exposure to the sun may bring on lupus skin lesions or trigger an internal response in susceptible people. Exactly why ultraviolet radiation has this effect isn't well understood, but scientists suspect that sunlight may cause skin cells to express certain proteins on their surface. Antibodies that are normally present in the body then latch onto these proteins, initiating an inflammatory response. Damaged skin cells also seem to die more frequently in people with lupus, leading to even more inflammation.
Certain prescription medications: Drug-induced lupus results from the long-term use of certain prescription drugs. Although many medications can potentially trigger lupus, examples of drugs most clearly linked with the disease include the antipsychotic chlorpromazine, high blood pressure medications such as hydralazine, the tuberculosis drug isoniazid and the heart medication procainamide, among others. It usually takes several months or years of therapy with these drugs before symptoms appear, and even then, only a small percentage of people will ever develop lupus.
Infection with Epstein-Barr virus: Almost everyone has been infected with a common human virus called Epstein-Barr virus. Epstein-Barr virus causes nonspecific signs and symptoms, such as fever and sore throat. Once the initial infection subsides, the virus remains dormant in the cells of your immune system unless something reactivates the virus. For reasons that aren't clear, recurrent Epstein-Barr infections seem to increase the risk of developing lupus.
Exposure to chemicals: It's difficult to prove that chemicals can cause or increase the risk of a disease. But some studies have shown that people who work in jobs that involve exposure to mercury and silica may have an increased risk of lupus.
Sex:Lupus is more common in women than in men.
When determining if someone has lupus, your physician has several diagnostic tools available to help her make a diagnosis. The most common include a complete medical history, physical examination, skin and kidney biopsies, as well as various lab tests, which might be referred to as bloodworak.
Medical history - Telling a doctor about your symptoms and other problems you have had can help him or her understand your situation. Your history can provide clues to your disease. Use the checklist at the end of this booklet to keep track of your symptoms. Share this checklist with your doctor. Ask your family or friends to help you with the checklist or come up with questions for your doctor.
Complete physical exam - The doctor will look for rashes and other signs that something is wrong.
Laboratory testing of blood and urine samples-Blood and urine samples often show if your immune system is overactive.
Skin or kidney biopsy - In a biopsy, tissue that is removed by a minor surgical procedure is examined under a microscope. Skin or kidney tissue examined in this way can show signs of an autoimmune disease.
Typical lab tests include:
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, but it may be elevated if you have lupus, another inflammatory condition or an infection.
Kidney and liver assessment: Blood tests can assess how well your kidneys and liver are functioning. Lupus can affect these organs.
Urinalysis: An examination of a sample of your urine may show an increased protein level or red blood cells in the urine, which may occur if lupus has affected your kidneys.
Antinuclear antibody (ANA) test: A positive test for the presence of these antibodies — produced by your immune system — indicates a stimulated immune system, which is common in lupus and other autoimmune diseases. A positive ANA doesn't always mean that you have lupus, however. ANA levels can be elevated if you have an infection or if you're taking certain medications. If you test positive for ANA, your doctor may advise more-specific antibody testing and refer you to a rheumatologist, a doctor who specializes in musculoskeletal and autoimmune disorders such as arthritis or lupus.
Complete blood count: This test measures the number of red blood cells, white blood cells and platelets as well as the amount of hemoglobin, a protein in red blood cells. Results may indicate you have anemia, which commonly occurs in lupus. A low white blood cell or platelet count may occur in lupus as well.
Chest X-ray: An image of your chest may reveal abnormal shadows that suggest fluid or inflammation in your lungs. It may also show an enlarged heart as a result of a buildup of fluid within the pericardium (pericardial effusion).
Electrocardiogram (ECG): This test measures the pattern of electrical impulses generated in your heart. It can help identify irregular rhythms or damage.
Syphilis test: A false-positive result on a syphilis test can indicate anti-phospholipid antibodies in your blood, another indication of lupus. The presence of anti-phospholipid antibodies has been associated with an increased risk of blood clots, strokes and recurrent miscarriages.
Treatment of lupus depends on how serious a patient's case is. Mild cases may involve rashes and moderate pain. These cases can be treated with nonsteroidal anti-inflammatory drugs, such as aspirin and ibuprofen, Motrin, Advil. More serious rashes and joint problems may be treated with drugs also used to treat malaria.
More serious symptoms may have to be treated with steroids. Steroids can reduce inflammation and swelling. They have some serious side effects, however, so they must be used with caution.
The most seriously ill patients may be treated with immunosuppressant drugs. Immunosuppressant drugs cause the immune system to shut down partially or completely. These drugs also have very serious side effects. With a weakened immune system, a patient is at risk for many other kinds of infections.
Other lupus treatments are designed for specific systems affected by the disease. For example, substances that thin the blood can be used if blood clots have formed. A person whose kidneys have begun to fail may require kidney dialysis or even a kidney transplantation. Kidney dialysis is a process in which a machine artificially cleanses a person's blood.
Lupus can cause skin rashes on any part of the body. One that often occurs on the face is called a butterfly rash.
Alternative Treatment: A number of alternative treatments have been suggested to help reduce the symptoms of lupus. These include acupuncture and massage for relieving the pain of sore joints and muscles. Patients can be taught to relax with techniques such as meditation and yoga. Hydrotherapy (water therapy) may also promote relaxation.
Proper nutrition may be an important factor in treating the symptoms of lupus. Some cases of the disease may be triggered by certain types of foods. Wheat, dairy products, and soy are the most common causes of the disease. Avoiding these foods may reduce the symptoms of lupus.
Some practitioners recommend nutritional supplements, such as vitamins B, C, and E, and minerals, such as magnesium, selenium, and zinc. They think these supplements may improve the general health of patients with lupus. Vitamin A can also be used to treat facial rashes.
Herbalists believe that certain herbs help relieve specific symptoms of lupus. They think that herbs can also help people develop a healthier outlook on life.
Treatment for aggressive lupus:
Life-threatening cases of lupus — those including kidney problems, inflammation in the blood vessels, and central nervous system problems, such as seizures — may require more aggressive treatment. In these cases, you and your doctor may consider:
Immunosuppressive drugs: Drugs that suppress the immune system may be helpful in serious cases of lupus, but can cause serious side effects. The most commonly used immunosuppressive drugs include cyclophosphamide (Cytoxan) and azathioprine (Imuran). The drug mycophenolate mofetil (CellCept), another immunosuppressant, can be used to treat lupus-related kidney problems. All three of these drugs can be taken orally, and cyclophosphamide can also be given intravenously. Side effects include an increased risk of infection, liver damage, infertility and an increased risk of cancer.
High-dose corticosteroids: High-dose corticosteroids can be taken orally or administered through a vein in your arm (intravenously). A high-dose regimen of corticosteroids may help control dangerous signs and symptoms quickly, but can also cause serious side effects, including infections, mood swings, high blood pressure and osteoporosis. To minimize side effects, your doctor will give you the lowest dose needed to control your signs and symptoms and then reduce the dosage over time.
High-dose corticosteroids can be combined with immunosuppressive drugs to reduce the dosage of each drug, which may reduce the risk of side effects. Sometimes, even with aggressive treatment, your kidneys may fail. In that case, you may need kidney dialysis or, if kidney failure is permanent, a kidney transplant.
Clinical trials: Researchers are developing new treatments for lupus in clinical trials. These studies give people with lupus the chance to try new treatments, but they don't guarantee a cure. If you're interested in a clinical trial, discuss the options with your doctor to determine the benefits and risks.
Treatments being studied in clinical trials include:
Dehydroepiandrosterone (DHEA): Some clinical trials have shown that a synthetic form of the hormone DHEA may improve quality of life in people with lupus, though others haven't found this. Your body uses DHEA to make male and female sex hormones. Researchers are conducting further clinical trials to better understand whether DHEA may be useful for people with lupus.
Rituximab (Rituxan): Rituximab decreases the number of B cells, a type of white blood cell, in your body and has shown some promise in treating lupus in people who haven't responded to other immunosuppressants. However, rituximab has been linked to a fatal brain infection in two people with lupus.
Stem cell transplant: A stem cell transplant uses your own adult stem cells to rebuild your immune system. Doctors hope this treatment can help people with life-threatening cases of lupus. Before a stem cell transplant you're given a drug that coaxes the adult stem cells out of your bone marrow and into your bloodstream. The stem cells are then filtered from your blood and frozen for later use. Strong immunosuppressive drugs are administered to wipe out your immune system. Then the adult stem cells are put back into your body where they can rebuild your immune system.
Medicine and medications:
Medication Choices: If you have mild disease or symptoms that affect your quality of life but don't have organ-threatening problems, your doctor may prescribe:
Antimalarial drugs such as hydroxychloroquine (Plaquenil).
Low-dose corticosteroids and/or topical corticosteroid creams or ointments.
Nonsteroidal anti-inflammatory drugs (NSAIDs), sometimes in combination with antimalarial drugs.
If you have more severe disease, your doctor may prescribe:
Immunosuppressive medications, such as azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil, or methotrexate.
Corticosteroids, such as prednisone.
If you have previously had blood clots in a vein or artery (venous or arterial thrombosis), or have antiphospholipid antibody syndrome, which increases your risk of developing blood clots, your doctor may prescribe a blood thinner (anticoagulant). This is especially important if you have already developed blood clots. If you have antiphospholipid antibody syndrome but have not had any blood clots, you may be able to take aspirin. Aspirin is a nonsteroidal anti-inflammatory drug (NSAID) that is sometimes used to slow blood clotting in antiphospholipid antibody syndrome.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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