Systemic lupus erythematosus (SLE) is a chronic disease that causes inflammation in connective tissues, such as cartilage and the lining of blood vessels, which provide strength and flexibility to structures throughout the body. The signs and symptoms of SLE vary among affected individuals, and can involve many organs and systems, including the skin, joints, kidneys, lungs, central nervous system, and blood-forming (hematopoietic) system. SLE is one of a large group of conditions called autoimmune disorders that occur when the immune system attacks the body's own tissues and organs.

SLE may first appear as extreme tiredness (fatigue), a vague feeling of discomfort or illness (malaise), fever, loss of appetite, and weight loss. Most affected individuals also have joint pain, typically affecting the same joints on both sides of the body, and muscle pain and weakness. Skin problems are common in SLE. A characteristic feature is a flat red rash across the cheeks and bridge of the nose, called a "butterfly rash" because of its shape. The rash, which generally does not hurt or itch, often appears or becomes more pronounced when exposed to sunlight. Other skin problems that may occur in SLE include calcium deposits under the skin (calcinosis), damaged blood vessels (vasculitis) in the skin, and tiny red spots called petechiae. Petechiae are caused by a shortage of cells involved in clotting (platelets), which leads to bleeding under the skin. Affected individuals may also have hair loss (alopecia) and open sores (ulcerations) in the moist lining (mucosae) of the mouth, nose, or, less commonly, the genitals.

About a third of people with SLE develop kidney disease (nephritis). Heart problems may also occur in SLE, including inflammation of the sac-like membrane around the heart (pericarditis) and abnormalities of the heart valves, which control blood flow in the heart. Heart disease caused by fatty buildup in the blood vessels (atherosclerosis), which is very common in the general population, is even more common in people with SLE. The inflammation characteristic of SLE can also damage the nervous system, and may result in abnormal sensation and weakness in the limbs (peripheral neuropathy); seizures; stroke; and difficulty processing, learning, and remembering information (cognitive impairment). Anxiety and depression are also common in SLE.

People with SLE have episodes in which the condition gets worse (exacerbations) and other times when it gets better (remissions). Overall, SLE gradually gets worse over time, and damage to the major organs of the body can be life-threatening.

Normal variations (polymorphisms) in many genes can affect the risk of developing SLE, and in most cases multiple genetic factors are thought to be involved. In rare cases, variants (also called mutations) in single genes cause SLE. Most of the genes associated with SLE are involved in immune system function, and changes in these genes likely affect proper targeting and control of the immune response. Sex hormones and a variety of environmental factors including viral infections, diet, stress, chemical exposures, and sunlight are also thought to play a role in triggering this complex disorder. About 10 percent of SLE cases are thought to be triggered by drug exposure, and more than 80 drugs that may be involved have been identified.

In people with SLE, cells that have undergone self-destruction (apoptosis) because they are damaged or no longer needed are not cleared away properly. The relationship of this loss of function to the cause or features of SLE is unclear. Researchers suggest that these dead cells may release substances that cause the immune system to react inappropriately and attack the body's tissues, resulting in the signs and symptoms of SLE.

Normal Function

The protein produced from the IRF5 gene, called interferon regulatory factor 5 (IRF5), acts as a transcription factor, which means that it attaches (binds) to specific regions of DNA and helps control the activity of certain genes. When a virus is recognized in the cell, the IRF5 gene is turned on (activated), which leads to the production of IRF5 protein. The protein binds to specific regions of DNA that regulate the activity of genes that produce interferons and other cytokines. Cytokines are proteins that help fight infection by promoting inflammation and regulating the activity of immune system cells. In particular, interferons control the activity of genes that help block the replication of viruses, and they stimulate the activity of certain immune system cells known as natural killer cells.

Health Conditions Related to Genetic Changes

Rheumatoid arthritis

Studies have associated normal variations in the IRF5 gene with an increased risk of rheumatoid arthritis. This condition causes chronic abnormal inflammation, primarily affecting the joints. Rheumatoid arthritis is an autoimmune disorders, which occur when the immune system malfunctions and attacks the body's tissues and organs.

There is some evidence that certain variations of the IRF5 gene are associated with increased activity of the gene and elevated cytokines. However, it is unknown what role, if any, these effects play in the increased risk of rheumatoid arthritis. Researchers believe that a combination of genetic and environmental factors may contribute to the development of these conditions.

Systemic lupus erythematosus

Studies have associated normal variations in the IRF5 gene with an increased risk of systemic lupus erythematosus. This is a chronic disease that causes inflammation in connective tissues, such as cartilage and the lining of blood vessels, which provide strength and flexibility to structures throughout the body. Systemic lupus erythematosus is an autoimmune disorders, which occur when the immune system malfunctions and attacks the body's tissues and organs.

There is some evidence that certain variations of the IRF5 gene are associated with increased activity of the gene and elevated cytokines. However, it is unknown what role, if any, these effects play in the increased risk of systemic lupus erythematosus. Researchers believe that a combination of genetic and environmental factors may contribute to the development of these conditions.

Systemic scleroderma

Several normal variations in the IRF5 gene have been associated with an increased risk of developing systemic scleroderma, which is an autoimmune disorder characterized by the buildup of scar tissue (fibrosis) in the skin and internal organs. Although the IRF5 gene is known to stimulate the immune system in response to viruses, it is unknown how the gene variations contribute to the increased risk of systemic scleroderma. Researchers believe that a combination of genetic and environmental factors may play a role in development of the condition.

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Other Names for This Gene

• IRF-5
• IRF5_HUMAN
• SLEB10

Genomic Location

Normal Function

The NCF2 gene provides instructions for making a protein called neutrophil cytosolic factor 2 (also known as p67-phox). This protein is one part (subunit) of a group of proteins that forms an enzyme complex called NADPH oxidase, which plays an essential role in the immune system. Specifically, NADPH oxidase is primarily active in immune system cells called phagocytes. These cells catch and destroy foreign invaders such as bacteria and fungi. NADPH oxidase is also thought to regulate the activity of immune cells called neutrophils. These cells play a role in adjusting the inflammatory response to optimize healing and reduce injury to the body.

The presence of foreign invaders stimulates phagocytes and triggers the assembly of NADPH oxidase. This enzyme participates in a chemical reaction that converts oxygen to a toxic molecule called superoxide. Superoxide is used to generate several other compounds, including hydrogen peroxide (a strong disinfectant) and hypochlorous acid (the active ingredient in bleach). These highly reactive, toxic substances are known as reactive oxygen species. Phagocytes use these substances to kill foreign invaders, preventing them from reproducing in the body and causing illness.

Health Conditions Related to Genetic Changes

Chronic granulomatous disease

More than 50 mutations in the NCF2 gene have been found to cause chronic granulomatous disease. People with this disorder are at increased risk of developing recurrent episodes of infection and inflammation due to a weakened immune system. Mutations in the NCF2 gene cause less than 5 percent of all cases of this condition. These mutations change single protein building blocks (amino acids) in the neutrophil cytosolic factor 2 protein, which cause the protein to be abnormally short and nonfunctional or alter its 3-dimensional structure. All of these mutations decrease the function of the neutrophil cytosolic factor 2 protein or prevent its production. Without this protein, NADPH oxidase cannot assemble or function properly. As a result, phagocytes are unable to produce reactive oxygen species to kill foreign invaders and neutrophil activity is not regulated. A lack of NADPH oxidase leaves affected individuals vulnerable to many types of infection and excessive inflammation.

Systemic lupus erythematosus

Studies suggest that certain normal variations in the NCF2 gene can increase the risk of a condition called systemic lupus erythematosus. This condition is one of a group of related diseases known as autoimmune disorders, which occur when the immune system malfunctions and attacks the body's tissues and organs. The variants associated with increased risk of systemic lupus erythematosus change single DNA building blocks (nucleotides) in the NCF2 gene. These changes are thought to result in the production of a neutrophil cytosolic factor 2 protein with an altered function that impairs the function of NADPH oxidase. As a result, fewer reactive oxygen species are produced when foreign invaders trigger an immune reaction. This lack of reactive oxygen species causes the body to overcompensate by activating more immune cells and producing more immune proteins. The overactive immune reaction increases the risk that the immune cells will attack the body's tissues and organs, causing systemic lupus erythematosus. Researchers believe that a combination of genetic and environmental factors play a role in development of this complex condition.

Other Names for This Gene

• NADPH oxidase activator 2
• NCF-2
• NCF2_HUMAN
• neutrophil cytosol factor 2
• NOXA2
• P67-PHOX
• P67PHOX

Genomic Location

Normal Function

The PTPN22 gene provides instructions for making a protein that belongs to the PTP (protein tyrosine phosphatases) family. PTP proteins play a role in regulating a process called signal transduction. In signal transduction, the protein relays signals from outside the cell to the cell nucleus. These signals instruct the cell to grow and divide or to mature and take on specialized functions.

The PTPN22 protein is involved in signaling that helps control the activity of immune system cells called T cells. T cells identify foreign substances and defend the body against infection.

Health Conditions Related to Genetic Changes

Vitiligo

Studies have associated the R620W variation in the PTPN22 gene with an increased risk of vitiligo, an autoimmune condition that results in patchy changes in skin coloring (pigmentation).

As with other autoimmune disorders, this variation likely affects the activity of the PTPN22 protein, making it more difficult for the body to control the immune system and prevent it from attacking its own tissues. While the pigment loss associated with vitiligo results from the immune system attacking pigment-producing cells (melanocytes) in the skin, it is unclear what specific circumstances trigger the immune system to do so. The condition probably results from a combination of genetic and environmental factors, most of which have not been identified.

Alopecia areata

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Autoimmune Addison disease

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Graves' disease

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Hashimoto's disease

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Idiopathic inflammatory myopathy

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Juvenile idiopathic arthritis

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Rheumatoid arthritis

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Systemic lupus erythematosus

MedlinePlus Genetics provides information about Systemic lupus erythematosus

Systemic scleroderma

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Type 1 diabetes

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Autoimmune disorders

Studies have associated a variation in the PTPN22 gene with an increased risk of several autoimmune disorders. Autoimmune disorders occur when the immune system malfunctions and attacks the body's tissues and organs. These disorders include type 1 diabetes, rheumatoid arthritis, Hashimoto thyroiditis, Graves disease, and systemic lupus erythematosus.

The PTPN22 gene variation associated with autoimmune disorders changes the protein building block (amino acid) arginine to the amino acid tryptophan at position 620 in the PTPN22 protein sequence, written as Arg620Trp or R620W. This variation likely affects the activity of the PTPN22 protein, making it more difficult for the body to control inflammation and prevent the immune system from attacking its own tissues.

Other Names for This Gene

• hematopoietic cell protein-tyrosine phosphatase 70Z-PEP
• lymphoid phosphatase
• lymphoid-specific protein tyrosine phosphatase
• LYP
• LYP1
• LYP2
• PEP
• PEST-domain phosphatase
• protein tyrosine phosphatase, non-receptor type 22 (lymphoid)
• protein tyrosine phosphatase, non-receptor type 8
• PTN22_HUMAN
• PTPN8
• tyrosine-protein phosphatase non-receptor type 22

Genomic Location

Normal Function

The STAT4 gene provides instructions for a protein that acts as a transcription factor, which means that it attaches (binds) to specific regions of DNA and helps control the activity of certain genes. The STAT4 protein is turned on (activated) by immune system proteins called cytokines, which are part of the inflammatory response to fight infection. When activated, the STAT4 protein increases the activity of genes that help immune cells called T-cells mature into specialized T-cells. These specialized T-cells, called Th1 cells, produce specific cytokines and stimulate other immune cells to get rid of foreign invaders (pathogens) in the cell.

Health Conditions Related to Genetic Changes

Systemic scleroderma

A normal variation in the STAT4 gene has been associated with an increased risk of developing systemic scleroderma, which is an autoimmune disorder characterized by the buildup of scar tissue (fibrosis) in the skin and internal organs. Although the STAT4 gene is known to stimulate the immune system in response to pathogens, it is unknown how the gene variation contributes to the increased risk of systemic scleroderma. Researchers believe that a combination of genetic and environmental factors may play a role in development of the condition.

Juvenile idiopathic arthritis

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Rheumatoid arthritis

Studies have associated a normal variation in the STAT4 gene with an increased risk of rheumatoid arthritis. This condition is an autoimmune disorder, which occurs when the immune system malfunctions and attacks the body's tissues and organs.

The variant associated with increased risk of rheumatoid arthritis changes a single DNA building block (nucleotide) in the STAT4 gene. It is unknown how the gene variation contributes to increased risk of this condition. Researchers believe that a combination of genetic and environmental factors may play a role in development of autoimmunity.

Systemic lupus erythematosus

Studies have associated a normal variation in the STAT4 gene with an increased risk of an autoimmune disorder called system lupus erythematosus.

The variant associated with increased risk of system lupus erythematosus changes a single nucleotide in the STAT4 gene. It is unknown how the gene variation contributes to increased risk of system lupus erythematosus. Researchers believe that a combination of genetic and environmental factors are likely involved in development of autoimmunity.

Autoimmune disorders

Studies have associated a normal variation in the STAT4 gene with an increased risk of an autoimmune disorder called Sjögren syndrome.

The variant associated with increased risk of this disorder changes a single nucleotide in the STAT4 gene. It is unknown how the gene variation contributes to increased risk of this condition. Researchers believe that a combination of genetic and environmental factors may play a role in disease development.

Other Names for This Gene

• SLEB11
• STAT4_HUMAN

Genomic Location

Normal Function

The TREX1 gene provides instructions for making the 3-prime repair exonuclease 1 enzyme. This enzyme is a DNA exonuclease, which means that it trims molecules of DNA by removing DNA building blocks (nucleotides) from the ends of the molecules. In this way, it breaks down unneeded DNA molecules or fragments that may be generated during copying (replication) of cells' genetic material in preparation for cell division. These fragments may also be generated during DNA repair, cell death (apoptosis), and other processes.

Health Conditions Related to Genetic Changes

Aicardi-Goutières syndrome

At least 82 mutations in the TREX1 gene have been identified in people with Aicardi-Goutières syndrome, a disorder that involves severe brain dysfunction (encephalopathy), skin lesions, and other health problems. Most of these mutations are believed to prevent the production of the 3-prime repair exonuclease 1 enzyme. Researchers suggest that the absence of this enzyme results in an accumulation of unneeded DNA and RNA in cells. These DNA and RNA molecules may be mistaken by cells for the genetic material of viral invaders, triggering immune system reactions that damage the brain, skin, and other organs and systems and result in the signs and symptoms of Aicardi-Goutières syndrome.

Systemic lupus erythematosus

Mutations in the TREX1 gene have also been identified in people with another disorder involving the immune system. This disorder is a chronic inflammatory disease called systemic lupus erythematosus (SLE). People with TREX1 gene mutations may develop a rare form of SLE called chilblain lupus that mainly affects the skin. Features of SLE, especially chilblain lupus, often also occur in people with Aicardi-Goutières syndrome (described above). It is likely that absence or impaired function of the 3-prime repair exonuclease 1 enzyme causes immune system dysfunction.

Other disorders

TREX1 gene mutations have also been found in people with a disorder called autosomal dominant retinal vasculopathy with cerebral leukodystrophy, which affects the brain and the blood vessels in the specialized light-sensitive tissue that lines the back of the eye (the retina).

As in Aicardi-Goutières syndrome, absence or impaired function of the 3-prime repair exonuclease 1 enzyme may cause immune system dysfunction that damages the brain, skin, blood vessels, and other parts of the body. It is not clear why mutations in the same gene cause several different disorders.

Other Names for This Gene

• 3' repair exonuclease 1
• 3'-5' exonuclease TREX1
• AGS1
• CRV
• deoxyribonuclease III, dnaQ/mutD-like
• DKFZp434J0310
• DNase III
• DRN3
• HERNS
• three prime repair exonuclease 1 isoform a
• three prime repair exonuclease 1 isoform b
• TREX1_HUMAN

Genomic Location

SLE and other autoimmune disorders tend to run in families, but the inheritance pattern is usually unknown. People may inherit a gene variation that increases or decreases the risk of SLE, but in most cases do not inherit the condition itself. Not all people with SLE have a gene variation that increases the risk, and not all people with such a gene variation will develop the disorder.

A rare, inherited form of SLE follows an autosomal recessive inheritance pattern, which means both copies of the gene in each cell have disease-causing variants. The parents of an individual with an autosomal recessive condition each carry one copy of the altered gene, but they typically do not show signs and symptoms of the condition.

Lupus can affect almost any organ in your body. The symptoms of lupus also differ from person to person. For example, one person with lupus may have swollen knees and fever. Another person may be tired all the time or have kidney trouble. Someone else may have rashes. Over time, new symptoms can develop or some symptoms may happen less often.

Lupus symptoms also usually come and go, meaning that you don’t have them all of the time. Lupus is a disease of flares (the symptoms worsen and you feel ill) and remissions (the symptoms improve and you feel better).

Lupus symptoms include:

• Muscle and joint pain. You may experience pain and stiffness, with or without swelling. This affects most people with lupus. Common areas for muscle pain and swelling include the neck, thighs, shoulders, and upper arms.
• Fever. A fever higher than 100 degrees Fahrenheit affects many people with lupus. The fever is often caused by inflammation or infection. Lupus medicine can help manage and prevent fever.
• Rashes. You may get rashes on any part of your body that is exposed to the sun, such as your face, arms, and hands. One common sign of lupus is a red, butterfly-shaped rash across the nose and cheeks.
• Chest pain. Lupus can trigger inflammation in the lining of the lungs. This causes chest pain when breathing deeply.
• Hair loss. Patchy or bald spots are common. Hair loss could also be caused by some medicines or infection.
• Sun or light sensitivity. Most people with lupus are sensitive to light, a condition called photosensitivity. Exposure to light can cause rashes, fever, fatigue, or joint pain in some people with lupus.
• Kidney problems. Half of people with lupus also have kidney problems, called lupus nephritis. Symptoms include weight gain, swollen ankles, high blood pressure, and decreased kidney function.
• Mouth sores. Also called ulcers, these sores usually appear on the roof of the mouth, but can also appear in the gums, inside the cheeks, and on the lips. They may be painless, or you may have soreness or dry mouth.
• Prolonged or extreme fatigue. You may feel tired or exhausted even when you get enough sleep. Fatigue can also be a warning sign of a lupus flare.
• Anemia. Fatigue could be a sign of anemia, a condition that happens when your body does not have red blood cells to carry oxygen throughout your body.
• Memory problems. Some people with lupus report problems with forgetfulness or confusion.
• Blood clotting. You may have a higher risk of blood clotting. This can cause blood clots in the legs or lungs, stroke, heart attack, or repeated miscarriages.
• Eye disease. You may get dry eyes, eye inflammation, and eyelid rashes.

Learn how to manage your lupus symptoms and prevent flares.

Lupus can be hard to diagnose because it has many symptoms that are often mistaken for symptoms of other diseases. Many people have lupus for a while before they find out they have it. If you have symptoms of lupus, tell your doctor right away.

No single test can tell if a person has lupus. But your doctor can find out if you have lupus in other ways, including:

• Medical history. Tell your doctor about your symptoms and other problems. Keep track of your symptoms by writing them down when they happen. Also, track how long they last.
• Family history of lupus or other autoimmune diseases. Tell your doctor if lupus or other autoimmune diseases run in your family.
• Complete physical exam. Your doctor will look for rashes and other signs that something is wrong.
• Blood and urine tests. The antinuclear antibody (ANA) test can show if your immune system is more likely to make the autoantibodies of lupus. Most people with lupus test positive for ANA. But, a positive ANA does not always mean you have lupus. If you test positive for ANA, your doctor will likely order more tests for antibodies that are specific to systemic lupus erythematosus (SLE).
• Skin or kidney biopsy. A biopsy is a minor surgery to remove a sample of tissue. The tissue is then viewed under a microscope. Skin and kidney tissue looked at in this way can show signs of an autoimmune disease.

Your doctor may use any or all of these tests to make your diagnosis. They also can help your doctor rule out other diseases that can be confused with lupus.

Talk to your doctor if you have lupus symptoms. Lupus is a chronic disease with no cure. This means that you can manage it with treatment, but it will not go away. Treatment can help improve your symptoms, prevent flares, and prevent other health problems often caused by lupus. Your treatment will depend on your symptoms and needs.

How is lupus treated?

There is no cure for lupus but treatments can help you feel better and improve your symptoms. Your treatment will depend on your symptoms and needs. The goals of treatment are to:

• Prevent flares
• Treat symptoms when they happen
• Reduce organ damage and other problems

Your treatment might include medicines to:

• Reduce swelling and pain
• Calm your immune system to prevent it from attacking the organs and tissues in your body
• Reduce or prevent damage to the joints
• Reduce or prevent organ damage

What types of medicines treat lupus?

Several different types of medicines treat lupus. Your doctors and nurses may change the medicine they prescribe for your lupus as your symptoms and needs change.

Types of medicines commonly used to treat lupus include:

• Nonsteroidal anti-inflammatory drugs (NSAIDs). Over-the-counter NSAIDs, such as ibuprofen and naproxen, help reduce mild pain and swelling in joints and muscles.
• Corticosteroids. Corticosteroids (prednisone) may help reduce swelling, tenderness, and pain. In high doses, they can calm the immune system. Corticosteroids, sometimes just called “steroids,” come in different forms: pills, a shot, or a cream to apply to the skin. Lupus symptoms usually respond very quickly to these powerful drugs. Once this has happened, your doctor will lower your dose slowly until you no longer need it. The longer a person uses these drugs, the harder it becomes to lower the dose. Stopping this medicine suddenly can harm your body.

• Antimalarial drugs. Medicines that prevent or treat malaria also treat joint pain, skin rashes, fatigue, and lung inflammation. Two common antimalarial medicines are hydroxychloroquine (Plaquenil) and chloroquine phosphate (Aralen). Studies found that taking antimalarial medicine can stop lupus flares and may help people with lupus live longer.
• BLyS-specific inhibitors. These drugs limit the amount of abnormal B cells (cells in the immune system that create antibodies) found in people with lupus. A common type of BLyS-specific inhibitor that treats lupus symptoms, belimumab, blocks the action of a specific protein in the body that is important in immune response.
• Immunosuppressive agents/chemotherapy. These medicines may be used in severe cases of lupus, when lupus affects major organs and other treatments do not work. These medicines can cause serious side effects because they lower the body’s ability to fight off infections.
• Other medicines. You may need other medicines to treat illnesses or diseases that are linked to your lupus — such as high blood pressure or osteoporosis. Many people with lupus are also at risk for blood clots, which can cause a stroke or heart attack. Your doctor may prescribe anticoagulants (“blood thinners”), such as warfarin or heparin, to prevent your blood from clotting too easily. You cannot take warfarin during pregnancy.

Talk to your doctor:

• About any side effects you may have
• If your medicines no longer help your symptoms
• If you have new symptoms
• If you want to become pregnant
• About any vitamins or herbal supplements you take — they might not mix well with medicines you use to treat lupus

Can I treat my lupus with alternative medicine?

Some people with lupus try creams, ointments, fish oil, or supplements they can buy without a prescription. Some people try homeopathy or see a chiropractor to care for their lupus. Some people with lupus who try these types of treatments say that they help.

Research studies have not shown any benefits to these types of treatments. And research studies have not been done to see if these treatments hurt people with lupus.

Talk to your doctor or nurse before trying any alternative medicine. Also, don’t stop or change your prescribed treatment without first talking to your doctor or nurse.

Will I need to see a special doctor for my lupus?

Maybe. Start by seeing your family doctor and a rheumatologist, a doctor who specializes in the diseases of joints and muscles such as lupus. Depending on your symptoms or whether your organs have been hurt by your lupus, you may need to see other types of doctors. These may include nephrologists, who treat kidney problems, and clinical immunologists, who treat immune system disorders.

Can I die from lupus?

Yes, lupus can cause death. But, thanks to new and better treatments, most people with lupus can expect to live long, healthy lives. The leading causes of death in people with lupus are health problems that are related to lupus, such as kidney disease, infections, and heart disease.

Work with your doctor to manage lupus. Take your medicine as your doctor tells you to and make healthy choices, such as not smoking, eating healthy foods, getting regular physical activity, and managing your weight. Learn more about managing Lupus.

What research is being done on lupus?

Lupus research focuses on:

• The genes that play a role in lupus and in the immune system
• Ways to change the immune system in people with lupus
• Different symptoms and effects of lupus in different racial and ethnic groups
• Things in the environment that may cause lupus
• The role of hormones in lupus
• Birth control pills and hormone therapy use in women with lupus
• Heart disease in people with lupus
• The causes of nervous system damage in people with lupus
• Treatments for lupus
• Treatments for organ damage caused by lupus, including stem cell transplantation
• Getting a better idea of how many people have lupus in the United States

What is systemic lupus erythematosus (SLE)?

Systemic lupus erythematosus (SLE) is the most common form of lupus. Lupus is different for each person and can affect different areas of the body, including:

• Skin.
• Joints.
• Heart.
• Lungs.
• Kidneys.
• Brain.

An autoimmune disease is a condition where your immune system—which is supposed to fight off viruses, bacteria, and infection—attacks its own tissues instead.

If you have lupus, you may have times of more symptoms (flares) and times of feeling better (remission). Lupus flares can be mild to serious, and they do not follow a pattern. However, with treatment, many people with lupus can manage the disease.

Anyone can get lupus, but women are more likely to get the disease than men are. Doctors and researchers are not sure what causes lupus, but they are working to learn more about what triggers the problems with the immune system.

What are the symptoms of SLE?

The symptoms of SLE can be different for everyone. They may include:

• Arthritis, causing painful and swollen joints and morning stiffness.
• Fevers.
• Feeling tired often.
• Rashes.
• Hair loss.
• Sores, which are usually painless, in the nose and mouth.
• Stomach pain.
• Change of color in the fingers and toes—blue-purplish, white, or red—from cold and stress.
• Swollen glands.
• Swelling in the legs or around the eyes.
• Pain when breathing deeply or lying down.
• Headaches, dizziness, depression, confusion, or seizures.

Your symptoms may be mild or severe; they may come and go; and they may affect one or several parts of your body. Some people with lupus start to have other problems with the kidneys, heart, or lungs.

How do doctors recognize SLE?

A rheumatologist is a doctor who specializes in treating diseases that affect the joints and muscles, like lupus.

Many people can have lupus for a long time before they find out they have it. This happens because many of the symptoms of lupus are also the same symptoms that happen with other disorders or diseases. That’s why it’s important to tell your doctor about your symptoms.

There isn’t just one test to diagnose lupus. Your doctor uses the information you tell them during your visit, along with a physical exam and the results of laboratory tests, to help diagnose lupus or decide if you have something else.

You may want to ask your primary care doctor for a referral to a rheumatologist. You may also see other types of doctors, depending on how lupus affects you.

How do doctors treat SLE?

Although there is no cure for lupus right now, doctors have many ways to manage the disease. Treatment depends on the symptoms you have. You may receive medications to help:

• Control pain.
• Lower a fever.
• Stop your immune system from being overactive.

No matter what treatment you receive, it is important that you have regular visits with your doctor to keep track of your symptoms and potential side effects of treatment. Never stop your medicines or treatments without speaking to your doctor. Download the PDF at the top of this page for a printable booklet that includes tools such as a medication tracker, symptom tracker, and daily activity tracker.

How to help manage SLE

Living with lupus can be hard, but a positive outlook is important. You can do several things to help you live with lupus. A good place to start managing your lupus is to work with your doctor and take your medications as directed. At times, you may feel sadness and anger. But keep in mind, many people with lupus live satisfying and productive lives. Most people with mild disease or who are in remission can usually participate in the same life activities they did before they were diagnosed.

Here are some tips to help you and your family manage lupus.

Learn about the signs of a flare

These signs may come and go. You may have swelling and rashes one week and no symptoms at all the next. You may find that your symptoms flare after you’ve been out in the sun, after a hard day at work, or at times when you are stressed.

Learning to recognize that a flare is coming can help you take steps to cope with it. Just before a flare, many people feel very tired or have pain, a rash, a fever, stomach discomfort, a headache, or joint swelling.

Steps to help with flares

• Taking steps to prevent flares can be helpful. You can:
• Talk with your doctor.
• Set realistic goals and priorities.
• Limit the time you spend in the sun.
• Eat a diet rich in fruits, vegetables, and whole grains.
• Manage your stress (see the "Cope With Stress" section below).
• Get enough rest and quiet time.
• Exercise moderately when possible.
• Ask for help from family and friends when you need it.

Cope with stress

Dealing with a long-lasting disease like lupus can be hard on your emotions. You might think that your friends, family, and coworkers do not understand how you feel. If you feel sad, depressed, or alone, consider:

• Seeking counseling from a mental health professional.
• Joining online and community support groups, and connecting with others on social media.
• Learning as much as you can about lupus and what you can do to feel better.
• Talking to your friends and family to help them understand what is happening to your body.
• Taking a break from focusing on the disease, and spending some time doing activities you enjoy.
• Meditating, reading, or deep breathing.

Exercising is another approach that can help you cope with stress and with lupus. Exercise can improve the strength and flexibility of your muscles, maintain the health of your joints, and increase your overall endurance. Be sure to talk with your doctor before starting any type of exercise program.

Pregnancy with lupus

Most women with lupus can have healthy pregnancies if the disease is under control. If you are planning to become pregnant, talk to your doctor so you can be as healthy as possible before becoming pregnant. Find an obstetrician who has experience working with women who have lupus.

SLE has been estimated to affect between 322,000 and 1.5 million people in the United States. The exact prevalence is difficult to determine because many of the signs and symptoms of SLE resemble those of other disorders. Diagnosis may be delayed for years, and the condition may never be diagnosed in some affected individuals. Females develop SLE about nine times more often than males. It is most common in younger women, peaking during the childbearing years; however, 20 percent of SLE cases occur in people over age 50.

For unknown reasons, in industrialized Western countries SLE has become 10 times more common over the past 50 years. The prevalence of SLE in Africa and Asia is believed to be much lower than in Western nations; however, in industrialized Western countries, people of African and Asian descent are two to four times more likely to develop SLE than are people of European descent. Researchers suggest that factors such as ethnic mixing, tobacco use in industrialized countries, and the different types of infections people acquire in different regions may contribute to these differences. For example malaria, which occurs often in tropical regions, is thought to be protective against SLE, while the Epstein-Barr virus, more common in the West, increases SLE risk.