Definition

By Mayo Clinic staff

Lupus is a chronic inflammatory disease that occurs when your body's immune system attacks your own tissues and organs. Inflammation caused by lupus can affect many different body systems, including your joints, skin, kidneys, blood cells, heart and lungs.

Lupus occurs more frequently in women, though it isn't clear why. Four types of lupus exist — systemic lupus erythematosus, discoid lupus erythematosus, drug-induced lupus erythematosus and neonatal lupus. Of these, systemic lupus erythematosus is the most common and serious form of lupus.

The outlook for people with lupus was once grim, but diagnosis and treatment of lupus has improved considerably. With treatment, most people with lupus can lead active lives.

No two cases of lupus are exactly alike. Signs and symptoms may come on suddenly or develop slowly, may be mild or severe, and may be temporary or permanent. Most people with lupus have mild disease characterized by episodes — called flares — when signs and symptoms get worse for a while, then improve or even disappear completely for a time.

The signs and symptoms of lupus that you experience will depend on which body systems are affected by the disease. But, in general, lupus signs and symptoms may include:

Fatigue
Fever
Weight loss or gain
Joint pain, stiffness and swelling
Butterfly-shaped rash (malar rash) on the face that covers the cheeks and bridge of the nose
Skin lesions that appear or worsen with sun exposure
Mouth sores
Hair loss (alopecia)
Fingers and toes that turn white or blue when exposed to cold or during stressful periods (Raynaud's phenomenon)
Shortness of breath
Chest pain
Dry eyes
Easy bruising
Anxiety
Depression
Memory loss

When to see a doctorIf you develop an unexplained rash, ongoing fever, persistent aching or fatigue, see your doctor.

If you've already been diagnosed with lupus, meet with your doctor on a regular basis so that your condition and treatment can be monitored. Make an appointment with your doctor if new symptoms arise.

Causes

By Mayo Clinic staff

Lupus is an autoimmune disease, which means that instead of just attacking foreign substances, such as bacteria and viruses, your immune system also turns against healthy tissue. This leads to inflammation and damage to various parts of the body, including the joints, skin, kidneys, heart, lungs, blood vessels and brain.

Doctors don't know what causes autoimmune diseases, such as lupus. It's likely that lupus results from a combination of your genetics and your environment. Doctors believe that you may inherit a predisposition to lupus, but not lupus itself. Instead, people with an inherited predisposition for lupus may only develop the disease when they come into contact with something in the environment that can trigger lupus, such as a medication or a virus.

Types of lupusFour types of lupus exist. Although similar, each type of lupus has a different prognosis and treatment.

Systemic lupus erythematosus can affect nearly any part of your body. Body systems most commonly involved include the skin, joints, lungs, kidneys and blood. When people talk about lupus, they're usually referring to systemic lupus erythematosus.
Discoid lupus erythematosus affects only the skin. People with discoid lupus, also called cutaneous lupus, experience a circular rash on the face, neck and scalp. A small number of people with discoid lupus may develop systemic lupus erythematosus, though it isn't possible to predict who will develop the more serious form of lupus.
Drug-induced lupus erythematosus occurs after you take certain prescription medications. Not everyone who takes these medications develops lupus. Drug-induced lupus affects a wide variety of body systems. Signs and symptoms usually go away when you stop taking the medication that caused your lupus.
Neonatal lupus is a rare form of lupus that affects newborn babies. A mother with certain antibodies that are linked to autoimmune diseases can pass them to the developing fetus — even if the mother has no signs or symptoms of an autoimmune disease. The antibodies can cause neonatal lupus. A baby with neonatal lupus may experience a rash in the weeks following birth. Neonatal lupus may last several months before disappearing. More-serious cases can cause a problem with the electrical system of the heart (congenital heart block).

Complications

By Mayo Clinic staff

Inflammation caused by lupus can affect many areas of your body, including your:

Kidneys. Lupus can cause serious kidney damage, and kidney failure is one of the leading causes of death among people with lupus. Up to three-quarters of people with lupus will develop kidney damage, usually during the first two years after diagnosis. However, with treatment, most people who develop lupus-related kidney problems can be effectively treated with medications. A blood test called serum creatinine level and urinalysis are used to monitor kidney function. Signs and symptoms of kidney problems may include generalized itching, chest pain, nausea, vomiting, leg swelling (edema) and weight gain.
Central nervous system (CNS). If your central nervous system is affected by lupus, you may experience headaches, dizziness, behavior changes, hallucinations and even seizures. As many as 80 percent of people with lupus may experience cognitive dysfunction, such as confusion, memory problems and difficulty expressing their thoughts.
Blood and blood vessels. Lupus may lead to blood problems, including anemia and increased risk of bleeding or blood clotting. It can also cause inflammation of the blood vessels (vasculitis), a serious complication responsible for 7 percent of lupus-related deaths.
Lungs. Having lupus increases your chances of developing an inflammation of the chest cavity lining (pleurisy) that can make breathing painful. You may also be more susceptible to a noninfectious form of pneumonia. Although a majority of people with lupus develop lung problems that can be detected by pulmonary tests, lung complications are rarely severe and often produce no symptoms.
Heart. Lupus can cause inflammation of your heart muscle (myocarditis and endocarditis), your arteries (coronary vasculitis) or heart membrane (pericarditis). Having lupus also greatly increases your risk of cardiovascular disease and heart attacks. Nearly 40 percent of people with lupus develop prematurely hardened arteries (atherosclerosis), compared with 15 percent of their peers who don't have lupus. Controlling high blood pressure and high blood cholesterol, not smoking, and getting regular exercise are essential to help reduce the risk of heart disease.
Infection. People with lupus are vulnerable to infection because both the disease and its treatments — corticosteroid and cytotoxic drugs, in particular — affect the immune system. And in a vicious cycle, infection can bring on a lupus flare, increasing the risk of infection even more. Infections that most commonly affect people with lupus include urinary tract infections; common respiratory infections, such as colds; yeast infections; salmonella; herpes and shingles. More-serious infections, such as pneumonia, account for about one-third of lupus-related deaths.
Cancer. Having lupus appears to increase your risk of cancer — especially non-Hodgkin's lymphoma, which affects the lymphatic system, and lung cancer. Immunosuppressant drugs that are sometimes used to treat lupus also can increase the risk of cancer. However, other than a slightly increased risk of death from non-Hodgkin's lymphoma or lung cancer, people with lupus are less likely to die of cancer than is the general population.
Bone tissue death (avascular necrosis). This occurs when the blood supply to a bone diminishes, often leading to tiny breaks in the bone and eventually to the bone's collapse. The hip joint is commonly affected, although avascular necrosis can occur in other bones as well. Avascular necrosis can be caused by lupus itself or by high doses of corticosteroids used to treat the disease. About 1 in 10 people with lupus develops symptoms of avascular necrosis.
Pregnancy complications. Women with lupus have an increased risk of miscarriage. Some women with lupus experience a flare during pregnancy. Lupus increases the risk of high blood pressure during pregnancy (preeclampsia) and preterm birth. About 2 in 10 women with lupus develop preeclampsia, and about 1 in 4 will deliver healthy babies prematurely. In addition, about 1 in 4 women with lupus who becomes pregnant will experience a miscarriage. To reduce the risk of these complications, doctors advise waiting to get pregnant until your disease has been under control or in remission for one year.

Preparing for your appointment

By Mayo Clinic staff

You're likely to start by seeing your family doctor or primary care provider. Because the symptoms of lupus can mimic so many other health problems, you may need patience while waiting for a diagnosis. Your doctor must rule out a number of other illnesses before diagnosing lupus.

Here's some information to help you prepare for your first appointment, and what to expect from your doctor.

What you can do

Write down your symptoms, including when you first noticed them and how often they occur.
Write down key personal information, including any recent changes or major stressors in your life.
Make a list of your key medical information, including any other conditions for which you're being treated and the names of any medications, vitamins or supplements you're taking. Your doctor will also want to know if you have any family history of lupus or other autoimmune disorders.
Take a family member or friend along, if possible. Sometimes it can be difficult to soak up all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
Write down questions to ask your doctor. Creating your list of questions in advance can help you make the most of your time with your doctor.

For lupus, some basic questions to ask your doctor include:

What are the possible causes of my symptoms or condition?
What tests do you recommend?
If these tests don't pinpoint the cause of my symptoms, what additional tests might I need?
Are there any treatments or lifestyle changes that might help my symptoms now?
Do I need to follow any restrictions while we're seeking a diagnosis?
Should I see a specialist?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment at any time that you don't understand something.

What to expect from your doctorYour doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to spend more time on. Your doctor may ask:

What are your symptoms? What part of your body is affected?
When did you first notice these symptoms?
Have your symptoms changed over time?
Do your symptoms seem to come and go?
Have you noticed anything that seems to trigger your symptoms?
Do your symptoms include extreme fatigue?
Have you had pain, stiffness or swelling in your joints?
Does sun exposure cause you to develop skin rashes?
Do your fingers or toes turn cold, pale or purple from cold or stress?
Do your symptoms include chest pain with deep breathing?
Have you experienced shortness of breath?
Have you had an unexplained fever?
Do your symptoms include any problems with memory or concentration?
How often do you feel anxious or depressed?
How much do your symptoms limit your ability to function at school, work or in personal relationships?
Have you been diagnosed with any other medical conditions?
Are you taking any medications, including over-the-counter and prescription drugs as well as vitamins and supplements?
Have any first-degree relatives — such as a parent or sibling — had lupus or another autoimmune disease?
Are you pregnant or do you plan to become pregnant?

What you can do in the meantime
While you're waiting for your appointment, get as much rest as your body seems to need and limit activities that wear you out. Try to reduce stress and schedule time only on activities that you enjoy and that help you manage stress. You may find that relaxation techniques such as yoga and meditation help improve your symptoms and your ability to cope.

Tests and diagnosis

By Mayo Clinic staff

Diagnosing lupus is difficult because signs and symptoms vary considerably from person to person. Signs and symptoms of lupus may change over time and overlap with those of many other disorders. For these reasons, doctors may not initially consider lupus until the signs and symptoms become more obvious. Even then, lupus can be challenging to diagnose because nearly all people with lupus experience fluctuations in disease activity. At times the disease may become severe and at other times subside completely.

American College of Rheumatology criteria for a lupus diagnosisThe American College of Rheumatology (ACR) has developed clinical and laboratory criteria to help physicians diagnose and classify lupus. If you have 4 of the 11 criteria at one time or individually over time, you probably have lupus. Your doctor may also consider the diagnosis of lupus even if you have fewer than four of these signs and symptoms. The criteria identified by the ACR include:

Face rash, which doctors call a malar rash, that is butterfly shaped and covers the bridge of the nose and spreads across the cheeks
Scaly rash, called a discoid rash, which appears as raised, scaly patches
Sun-related rash, which appears after exposure to sunlight
Mouth sores, which are usually painless
Joint pain and swelling that occurs in two or more joints
Swelling of the linings around the lungs or the heart
Kidney disease
A neurological disorder, such as seizures or psychosis
Low blood counts, such as low red blood count, low platelet count (thrombocytopenia), or a low white cell count (leukopenia)
Positive antinuclear antibody tests, which indicate that you may have an autoimmune disease
Other positive blood tests that may indicate an autoimmune disease, such as a positive double-stranded anti-DNA test, positive anti-Sm test, positive anti-phospholipid antibody test or false-positive syphilis test

Laboratory testsYour doctor may order blood and urine tests to determine your diagnosis, including:

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.
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.
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.

Treatments and drugs

By Mayo Clinic staff

Treatment for lupus depends on your signs and symptoms. Determining whether your signs and symptoms should be treated and what medications to use requires a careful discussion of the benefits and risks with your doctor. As your signs and symptoms flare and subside, you and your doctor may find that you'll need to change medications or dosages.

Common medications used to treat lupusThree types of drugs are commonly used to treat lupus when your signs and symptoms are mild or moderate. More aggressive lupus may require more aggressive drugs. In general, when first diagnosed with lupus, your doctor may discuss these medications:

Nonsteroidal anti-inflammatory drugs. Aspirin or other nonsteroidal anti-inflammatory drugs (NSAIDs), such as naproxen sodium (Aleve) and ibuprofen (Advil, Motrin, others), may be used to treat a variety of signs and symptoms associated with lupus. NSAIDs are available over-the-counter, or stronger versions can be prescribed by your doctor. Check with your doctor before taking over-the-counter NSAIDs because some have been associated with serious side effects in people with lupus. Side effects of NSAIDs include stomach bleeding, kidney problems and an increased risk of heart problems.
Antimalarial drugs. Although there's no known relationship between lupus and malaria, these medications have proved useful in treating signs and symptoms of lupus. Antimalarials may also prevent flares of the disease. Hydroxychloroquine (Plaquenil) is the most commonly prescribed antimalarial. Side effects of antimalarial drugs include vision problems and muscle weakness.
Corticosteroids. These drugs counter the inflammation of lupus, but can have serious long-term side effects, including weight gain, easy bruising, thinning bones (osteoporosis), high blood pressure, diabetes and increased risk of infection. The risk of side effects increases with higher doses and longer term therapy. To help reduce these risks, your doctor will try to find the lowest dose that controls your symptoms and prescribe corticosteroids for the shortest possible time. Taking the drug every other day also can help reduce side effects. Corticosteroids are sometimes combined with another medication to help reduce the dose, and therefore the toxicity, of both drugs. Taking calcium and vitamin D supplements while using corticosteroids can reduce the risk of osteoporosis.

Treatment for specific signs and symptomsWhat treatments you may consider depend on your signs and symptoms. Treatments for specific signs and symptoms include:

Joint pain and swelling. Pain in your joints may be initially controlled with NSAIDs. If you experience more significant joint pain, you and your doctor may consider antimalarial drugs or corticosteroids.
Skin rashes. Avoid skin rashes by staying out of the sun, wearing sunblock year-round and keeping your skin covered. Despite your best attempts to avoid the sun, even indoor fluorescent lighting can trigger skin rashes in people with lupus. Skin rashes are sometimes treated with topical corticosteroids. These creams are applied to the affected area to reduce the inflammation in your skin cells. Oral steroids or antimalarial drugs also can be used.
Fatigue. Fatigue is treated by determining the underlying cause. Your fatigue may be caused by difficulty sleeping, depression or poorly controlled pain. In these cases, your doctor would treat the underlying cause. If you and your doctor can't determine a cause for your fatigue, you may consider medications, such as corticosteroids and antimalarial drugs.
Swelling around the heart and lungs. Swelling around your heart and lungs that causes chest pain may be controlled by NSAIDs, antimalarial drugs or corticosteroids.

Treatment for aggressive lupusLife-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:

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.
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, Azasan). Less commonly, the immune protein gamma globulin and the disease-modifying anti-rheumatic drug methotrexate — normally used to treat rheumatic arthritis — may be used to control severe, treatment-resistant symptoms. The drug mycophenolate (CellCept), another immunosuppressant, can be used to treat lupus-related kidney problems. Depending on the drug, immunosuppressive medications may be taken orally or intravenously. Side effects include an increased risk of infection, liver damage, infertility and an increased risk of cancer.

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 trialsResearchers are studying many 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:

Belimumab (Benlysta). Phase 3 clinical trial results released in July 2009 indicated that this drug, when added to a standard lupus treatment regimen, significantly improved symptoms in the majority of participants. Belimumab is a monoclonal antibody, a laboratory-produced molecule that mimics the disease-fighting properties of your body's natural, immune system antibodies. Results from another large, stage 3 clinical trial of this drug are expected in late 2009.
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.
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 may benefit people who haven't responded to other immunosuppressants. However, study results have been inconsistent and more research is needed to understand whether this drug is useful in the treatment of lupus.

Lifestyle and home remedies

By Mayo Clinic staff

Take steps to care for your body if you have lupus. Simple steps can help you prevent lupus flares and, should they occur, better cope with the signs and symptoms you experience. Try to:

Get adequate rest. People with lupus often experience persistent fatigue that's different from normal tiredness and that isn't necessarily relieved by rest. For that reason, it can be hard to judge when you need to slow down. Get plenty of sleep a night and naps or breaks during the day as needed. Friends and family members need to understand and respect your need for rest.
Be sun smart. Because ultraviolet light can trigger a flare, wear protective clothing, such as a hat, long-sleeved shirt and long pants, and use sunscreens with a sun protection factor (SPF) of at least 15 every time you go outside, even if it's just a quick trip to the mailbox. Be sure that your ears, scalp and the backs of your hands are protected. Avoid tanning beds and stay out of the sun entirely when it's strongest, from 10 a.m. to 4 p.m. if possible. Because fluorescent and halogen lights also can emit ultraviolet (UV) rays and thus aggravate lupus, you may need to wear sunscreen and protective clothing indoors or use plastic devices that block UV emissions from indoor lights.
Get regular exercise. Exercise can help you recover from a flare, reduce your risk of heart attack, help fight depression and promote general well-being. Exercise as much as your body allows — aim for 30 total minutes of activity most days of the week, which can be divided up throughout the day. You'll likely feel fatigued and not up to exercising sometimes, and that's OK. Rest when you need to. Time outdoor activities so that you avoid the sun when it's most intense, and if you're having a flare, stay out of the sun entirely.
Don't smoke. Smoking increases your risk of cardiovascular disease and can worsen the effects of lupus on your heart and blood vessels.
Eat a healthy diet. A healthy diet emphasizes fruits, vegetables and whole grains. Sometimes you may have dietary restrictions, especially if you have high blood pressure, kidney damage or gastrointestinal problems. And although no specific foods have been shown to cause or worsen lupus, it's best to avoid any food that seems to make your symptoms worse.

Information copied from the Mayo Clinic web site