Lupus - Systemic Lupus Erythematosus (SLE)
Lupus is an autoimmune diseases that leads to inflammation and damage of parts of the body, including the joints, skin, kidneys, heart, lungs, blood vessels, and brain.
Symptoms of lupus vary widely, but the most common symptoms include extreme fatigue, painful or swollen joints (arthritis), unexplained fever, skin rashes, and kidney problems. Symptoms of lupus often come and go in cycles called flares and remissions.
At present, there is no cure for lupus. However, lupus can often be effectively controlled with medications and most people with the disease can lead active, healthy lives.
Different Types of Lupus
- Systemic lupus erythematosus (SLE) is the form of the disease that most people are referring to when they say "lupus." The word "systemic" means the disease can affect many parts of the body. The symptoms of SLE may be mild or serious. Although SLE usually first affects people between the ages of 15 and 45 years, it can occur in childhood or later in life as well.
- Discoid lupus erythematosus is a chronic skin disorder in which a red, raised rash appears on the face, scalp, or elsewhere. The raised areas may become thick and scaly and may cause scarring. The rash may last for days or years and may recur. A small percentage of people with discoid lupus have or develop SLE later.
- Subacute cutaneous lupus erythematosus refers to skin lesions that appear on parts of the body exposed to sun. The lesions do not result in permanent scars.
- Drug-induced lupus is a form of lupus caused by medications. Many different drugs can cause drug-induced lupus. Symptoms are similar to those of SLE (arthritis, rash, fever, and chest pain) and they typically go away completely when the drug is stopped. The kidneys and brain are rarely involved.
- Neonatal lupus is a rare disease that can occur in newborn babies of women with SLE, Sjögren's syndrome, or no disease at all. It is important for women with SLE or other related autoimmune disorders to be under a doctor's care during pregnancy. Physicians can now identify mothers at highest risk for complications, allowing for prompt treatment of the infant at or before birth. SLE can also flare during pregnancy, and prompt treatment can keep the mother healthier longer.
Causes of Lupus
Lupus is a complex disease, and its cause is unknown. It is likely that a combination of genetic, environmental, and possibly hormonal factors work together to cause the disease. The fact that lupus can run in families indicates that its development has a genetic basis. Recent research suggests that genetics plays an important role; however, no specific "lupus gene" has been identified yet.
Scientists believe that genes alone do not determine who gets lupus, and that other factors also play a role. Some of the factors scientists are studying include sunlight, stress, certain drugs, and infectious agents such as viruses.
It is likely that a combination of factors work together to cause the disease. In lupus, the body's immune system does not work as it should. A healthy immune system produces proteins called antibodies and specific cells called lymphocytes that help fight and destroy viruses, bacteria, and other foreign substances that invade the body. In lupus, the immune system produces antibodies against the body's healthy cells and tissues. These antibodies, called autoantibodies, contribute to the inflammation of various parts of the body and can cause damage to organs and tissues.
The most common type of autoantibody that develops in people with lupus is called an antinuclear antibody (ANA) because it reacts with parts of the cell's nucleus (command center). Doctors and scientists do not yet understand all of the factors that cause inflammation and tissue damage in lupus, and researchers are actively exploring them.
Symptoms of Lupus
Each person with lupus has slightly different symptoms that can range from mild to severe and may come and go over time.
Common symptoms of lupus
- Painful or swollen joints and muscle pain
- Unexplained fever
- Red rashes, most commonly on the face (sometimes called the "butterfly rash" for its shape)
- Chest pain upon deep breathing
- Unusual loss of hair
- Pale or purple fingers or toes from cold or stress (Raynaud's phenomenon)
- Sensitivity to the sun
- Swelling (edema) in legs or around eyes
- Mouth ulcers
- Swollen glands
- Extreme fatigue
New symptoms may continue to appear years after the initial diagnosis, and different symptoms can occur at different times. In some people with lupus, only one system of the body, such as the skin or joints, is affected. Other people experience symptoms in many parts of their body. Just how seriously a body system is affected varies from person to person.
The following systems in the body also can be affected by lupus.
- Kidneys: Inflammation of the kidneys (nephritis) can impair their ability to get rid of waste products and other toxins from the body effectively. There is usually no pain associated with kidney involvement, although some patients may notice swelling in their ankles. Most often, the only indication of kidney disease is an abnormal urine or blood test. Because the kidneys are so important to overall health, lupus affecting the kidneys generally requires intensive drug treatment to prevent permanent damage.
- Lungs: Some people with lupus develop pleuritis, an inflammation of the lining of the chest cavity that causes chest pain, particularly with breathing. Patients with lupus also may get pneumonia.
- Central nervous system: In some patients, lupus affects the brain or central nervous system. This can cause headaches, dizziness, memory disturbances, vision problems, seizures, stroke, or changes in behavior.
- Blood vessels: Blood vessels may become inflamed (vasculitis), affecting the way blood circulates through the body. The inflammation may be mild and may not require treatment or may be severe and require immediate attention.
- Blood: People with lupus may develop anemia, leukopenia (a decreased number of white blood cells), or thrombocytopenia (a decrease in the number of platelets in the blood, which assist in clotting). Some people with lupus may have an increased risk for blood clots.
- Heart: In some people with lupus, inflammation can occur in the heart itself (myocarditis and endocarditis) or the membrane that surrounds it (pericarditis), causing chest pains or other symptoms. Lupus can also increase the risk of atherosclerosis (hardening of the arteries).
Diagnosis of Lupus
Diagnosing lupus can be difficult. It may take months or even years for doctors to piece together the symptoms to diagnose this complex disease accurately.
No single test can determine whether a person has lupus, but several laboratory tests may help the doctor to make a diagnosis. The most useful tests identify certain autoantibodies often present in the blood of people with lupus. For example, the antinuclear antibody (ANA) test is commonly used to look for autoantibodies that react against components of the nucleus, or "command center," of the body's cells. Most people with lupus test positive for ANA; however, there are a number of other causes of a positive ANA besides lupus, including infections, other autoimmune diseases, and occasionally as a finding in healthy people. The ANA test simply provides another clue for the doctor to consider in making a diagnosis.
In addition, there are blood tests for individual types of autoantibodies that are more specific to people with lupus, although not all people with lupus test positive for these and not all people with these antibodies have lupus. These antibodies include anti-DNA, anti-Sm, anti-RNP, anti-Ro (SSA), and anti-La (SSB). The doctor may use these antibody tests to help make a diagnosis of lupus.
Some tests are used less frequently but may be helpful if the cause of a person's symptoms remains unclear. The doctor may order a biopsy of the skin or kidneys if those body systems are affected. Some doctors may order a test for anticardiolipin (or antiphospholipid) antibody. The presence of this antibody may indicate increased risk for blood clotting and increased risk for miscarriage in pregnant women with lupus. Again, all these tests merely serve as tools to give the doctor clues and information in making a diagnosis. The doctor will look at the entire picture—medical history, symptoms, and test results—to determine if a person has lupus.
Other laboratory tests are used to monitor the progress of the disease once it has been diagnosed. A complete blood count, urinalysis, blood chemistries, and the erythrocyte sedimentation rate (ESR) test can provide valuable information. Another common test measures the blood level of a group of substances called complement. People with lupus often have increased ESRs and low complement levels, especially during flares of the disease. X-rays and other imaging tests can help doctors see the organs affected by SLE.
Treatment of Lupus
Once lupus has been diagnosed, the doctor will develop a treatment plan based on the patient's age, sex, health, symptoms, and lifestyle. Treatment plans are tailored to the individual's needs and may change over time. In developing a treatment plan, the doctor has several goals: to prevent flares, to treat them when they do occur, and to minimize organ damage and complications. The doctor and patient should reevaluate the plan regularly to ensure it is as effective as possible.
For people with joint or chest pain or fever, drugs that decrease inflammation, called nonsteroidal anti-inflammatory drugs (NSAIDs), are often used. While some NSAIDs, such as ibuprofen and naproxen, are available over the counter, a doctor's prescription is necessary for others. NSAIDs may be used alone or in combination with other types of drugs to control pain, swelling, and fever. Even though some NSAIDs may be purchased without a prescription, it is important that they be taken under a doctor's direction. Common side effects of NSAIDs can include stomach upset, heartburn, diarrhea, and fluid retention. Some people with lupus also develop liver, kidney, or even neurological complications, making it especially important to stay in close contact with the doctor while taking these medications.
Antimalarials are another type of drug commonly used to treat lupus. These drugs were originally used to treat malaria, but doctors have found that they also are useful for lupus. A common antimalarial used to treat lupus is hydroxychloroquine (Plaquenil). It may be used alone or in combination with other drugs and generally is used to treat fatigue, joint pain, skin rashes, and inflammation of the lungs. Clinical studies have found that continuous treatment with antimalarials may prevent flares from recurring. Side effects of antimalarials can include stomach upset and, extremely rarely, damage to the retina of the eye.
The mainstay of lupus treatment involves the use of corticosteroid hormones, such as prednisone (Deltasone®), hydrocortisone, methylprednisolone (Medrol®), and dexamethasone (Decadron®, Hexadrol®). Corticosteroids are related to cortisol, which is a natural anti-inflammatory hormone. They work by rapidly suppressing inflammation. Corticosteroids can be given by mouth (oral corticosteroids), in creams applied to the skin (topical corticosteroids), or by injection. Because they are potent drugs, the doctor will seek the lowest dose with the greatest benefit. Short-term side effects of corticosteroids include swelling, increased appetite, and weight gain. These side effects generally stop when the drug is stopped. It is dangerous to stop taking corticosteroids suddenly, so it is very important that the doctor and patient work together in changing the corticosteroid dose. Sometimes doctors give very large amounts of corticosteroid by vein over a brief period of time (days) ("bolus" or "pulse" therapy). With this treatment, the typical side effects are less likely and slow withdrawal is unnecessary.
Long-term side effects of corticosteroids can include stretch marks on the skin, weakened or damaged bones (osteoporosis and osteonecrosis), high blood pressure, damage to the arteries, high blood sugar (diabetes), infections, and cataracts. Typically, the higher the dose and the longer they are taken, the greater the risk and severity of side effects. Researchers are working to develop ways to limit or offset the use of corticosteroids. For example, corticosteroids may be used in combination with other, less potent drugs, or the doctor may try to slowly decrease the dose once the disease is under control. People with lupus who are using corticosteroids should talk to their doctors about taking supplemental calcium and vitamin D or other drugs to reduce the risk of osteoporosis (weakened, fragile bones).
It is dangerous to stop taking corticosteroids suddenly, so it is very important that the doctor and patient work together in changing the dose.
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 (Cytoxan®) and mycophenolate mofetil (CellCept®), restrain the overactive immune system by blocking the production of immune cells. These drugs may be given by mouth or by infusion (dripping the drug into the vein through a small tube). Side effects may include nausea, vomiting, hair loss, bladder problems, decreased fertility, and increased risk of cancer and infection. The risk for side effects increases with the length of treatment. As with other treatments for lupus, there is a risk of relapse after the immunosuppressives have been stopped.
Monoclonal antibodies (mAbs)
Benlysta® (belimumab) is the first and only drug specifically developed for and approved to treat lupus. It was developed to disrupt activation of B lymphocytes by interfering with BLyS, a protein required for B cell activity. Benlysta®
In some patients, methotrexate (Folex®, Mexate®, Rheumatrex®), a disease-modifying antirheumatic drug, may be used to help control the disease. Working closely with the doctor helps ensure that treatments for lupus are as successful as possible. Because some treatments may cause harmful side effects, it is important to report any new symptoms to the doctor promptly. It is also important not to stop or change treatments without talking to the doctor first.
Alternative and complementary therapies
Because of the nature and cost of the medications used to treat lupus and the potential for serious side effects, many patients seek other ways of treating the disease. Some alternative approaches people have tried include special diets, nutritional supplements, fish oils, ointments and creams, chiropractic treatment, and homeopathy. Although these methods may not be harmful and may be associated with symptomatic or psychosocial benefit, no research to date shows that they affect the disease process or prevent organ damage. Some alternative or complementary approaches may help the patient cope or reduce some of the stress associated with living with a chronic illness. If the doctor feels the approach has value and will not be harmful, it can be incorporated into the patient's treatment plan. However, it is important not to neglect regular health care or treatment of serious symptoms. An open dialogue between the patient and physician about the relative values of complementary and alternative therapies allows the patient to make an informed choice about treatment options.
Quality of Life with Lupus
Despite the symptoms of lupus and the potential side-effects of treatment, people with lupus can maintain a high quality of life overall. One key to managing lupus is to understand the disease and its impact. Learning to recognize the warning signs of a flare can help the patient take steps to ward it off or reduce its intensity. Many people with lupus experience increased fatigue, pain, a rash, fever, abdominal discomfort, headache, or dizziness just before a flare. Developing strategies to prevent flares can also be helpful, such as learning to recognize your warning signals and maintaining good communication with your doctor.
It is also important for people with lupus to receive regular health care, instead of seeking help only when symptoms worsen. Results from a regular medical exams and laboratory work allow the doctor to note any changes and to identify and treat flares early. The treatment plan, which is tailored to the individual's specific needs and circumstances, can be adjusted accordingly. If new symptoms are identified early, treatments may be more effective. Other concerns also can be addressed at regular checkups. The doctor can provide guidance about such issues as the use of sunscreens, stress reduction, and the importance of structured exercise and rest, as well as birth control and family planning. Because people with lupus can be more susceptible to infections, the doctor may recommend yearly influenza vaccinations or pneumococcal vaccinations for some patients.
Women with lupus should receive regular preventive health care, such as gynecological and breast examinations. Men with lupus should have the prostate-specific antigen (PSA) test. Both men and women need to have their blood pressure and cholesterol checked on a regular basis. If a person is taking corticosteroids or antimalarial medications, an eye exam should be done at least yearly to screen for and treat eye problems.
Staying healthy requires extra effort and care for people with lupus, so it becomes especially important to develop strategies for maintaining wellness. Wellness involves close attention to the body, mind, and spirit. One of the primary goals of wellness for people with lupus is coping with the stress of having a chronic disorder. Effective stress management varies from person to person. Some approaches that may help include exercise, relaxation techniques such as meditation, and setting priorities for spending time and energy.
Developing and maintaining a good support system is also important. A support system may include family, friends, medical professionals, community organizations, and support groups. Participating in a support group can provide emotional help, boost self-esteem and morale, and help develop or improve coping skills.
Warning signs of a lupus flare
- Increased fatigue
- Abdominal discomfort
Preventing a lupus flare
- Learn to recognize your warning signals.
- Maintain good communication with your doctor.
- Learning more about lupus may also help. Studies have shown that patients who are well-informed and participate actively in their own care experience less pain, make fewer visits to the doctor, build self-confidence, and remain more active.
Tips for working with your doctor
- Seek a health care provider who is familiar with SLE and who will listen to and address your concerns.
- Provide complete, accurate medical information.
- Make a list of your questions and concerns in advance.
- Be honest and share your point of view with the health care provider.
- Ask for clarification or further explanation if you need it.
- Talk to other members of the health care team, such as nurses, therapists, or pharmacists.
- Do not hesitate to discuss sensitive subjects (for example, birth control or intimacy) with your doctor.
- Discuss any treatment changes with your doctor before making them.
Considerations for Women with Lupus During Pregnancy
Although a lupus pregnancy is considered high risk, most women with lupus carry their babies safely to the end of their pregnancy. Women with lupus have a higher rate of miscarriage and premature births compared with the general population. In addition, women who have antiphospholipid antibodies are at a greater risk of miscarriage in the second trimester because of their increased risk of blood clotting in the placenta. Lupus patients with a history of kidney disease have a higher risk of preeclampsia (hypertension with a buildup of excess watery fluid in cells or tissues of the body). Pregnancy counseling and planning before pregnancy are important. Ideally, a woman should have no signs or symptoms of lupus and be taking no medications for at least 6 months before she becomes pregnant.
Some women may experience a mild to moderate flare during or after their pregnancy; others do not. Pregnant women with lupus, especially those taking corticosteroids, also are more likely to develop high blood pressure, diabetes, hyperglycemia (high blood sugar), and kidney complications, so regular care and good nutrition during pregnancy are essential. It is also advisable to have access to a neonatal (newborn) intensive care unit at the time of delivery in case the baby requires special medical attention.