Lupus is an inflammatory disease that can involve many parts of the body, including the skin, joints, kidneys, blood vessels, heart, lungs and brain. In many patients the disease is mild, but in others it can lead to life-threatening complications.

Lupus is an autoimmune disease, meaning the patient's hyperactive immune system mistakenly attacks the body's own tissues. The most common and most serious form is called systemic lupus erythematosus (SLE). Other types of lupus include a temporary form brought on by certain drugs, a skin disease called discoid (cutaneous) lupus and a rare form that affects newborns.

Lupus varies widely in how it affects individuals. SLE patients typically experience long periods of remission between occasional flare-ups. Individuals may experience rashes, arthritis-like joint pain, fatigue, fever or other symptoms. Possible complications include kidney damage, osteoporosis, pregnancy difficulties or stroke.

The cause of SLE is unknown. Heredity and the influence of hormones may predispose a person to lupus, which may be set off by environmental triggers such as viruses or silica dust. Diagnosis is difficult and may take years because the disease can be mistaken for many other conditions and cannot be detected with any single test. A butterfly-shaped rash on the face is a common sign of lupus.

Drug-induced lupus can be resolved by discontinuing the medication, but there is no known cure for other types of lupus. However, most patients are able to manage the disease and live long, productive lives. Treatments include medications such as aspirin and corticosteroids. Exercise, stress management, relaxation techniques and rest help patients cope with flare-ups.

Ninety percent of lupus patients are women, according to the U.S. National Institutes of Health. The disease usually develops between ages 15 and 45 and is more common in blacks, Asians, Hispanics and American Indians than in whites.

Lupus is an inflammatory disease that can involve many parts of the body, including the joints, skin, kidneys, blood, blood vessels, heart, lungs and nervous system. The condition can range from mild to life-threatening.

Lupus is an autoimmune disorder, meaning the body mistakenly attacks itself. The immune system helps protect the body by making antibodies, which are cells that attack foreign substances such as viruses and bacteria. Sometimes, however, this system malfunctions and creates abnormal autoantibodies that attack the body's own tissues. This causes inflammation, vasculitis (damage to blood vessels) and organ damage due to a buildup of immune system cells.

Like many autoimmune diseases, lupus is also categorized as a connective tissue disorder because of its effect on bone, cartilage and other connective tissues. About 10 percent of lupus patients have symptoms typical of other connective tissue diseases such as rheumatoid arthritis, myositis, scleroderma or Sjogren's syndrome, according to the American College of Rheumatology. Doctors sometimes refer to this as overlap syndrome or mixed connective tissue disease.

Estimates of the prevalence of lupus vary. The Lupus Foundation of America says there are at at least 1.5 million to 2 million Americans with lupus, most of whom have the most serious form, systemic lupus erythematosus (SLE) However, the Centers for Disease Control and Prevention (CDC) states that the number of Americans with SLE can be conservatively estimated at 322,000 to possibly more than 1 million.

SLE lasts the rest of a person’s life, typically with long periods of good health (remissions) interrupted by occasional exacerbations (flares). Lupus is not contagious, meaning it isn't spread from one person to another. There is no known cure or prevention, but there are many ways for people to cope with the condition, such as exercise, stress management, rest and getting educated about the disease. Much research is being conducted to improve the treatment of lupus, including improved diagnostic methods, new medications and a vaccine.

Ninety percent of lupus patients are women, according to the U.S. National Institutes of Health (NIH). The disease usually develops between ages 15 and 45. It can also develop during childhood and in middle and old age.

Lupus is more common and more severe in blacks, Asians, Hispanics and American Indians than in whites, probably because of genetics. Black women are three times more likely than white women to develop lupus, according to the CDC. Scientists have linked a gene to the higher risk of kidney disease in black patients with SLE.

About 15 percent of SLE patients develop the disease after age 55, according to the Lupus Foundation of America. This late-onset SLE is more common in whites, includes a slightly higher proportion of men than earlier-onset SLE and is typically milder and easier to manage.

Lupus varies greatly in how it affects people. Some individuals experience few problems, but others suffer one or more serious complications. Joint pain and stiffness are common, but unlike typical forms of arthritis, deformities seldom develop in the joints. Potential complications of SLE include:

• Osteoporosis. As with lupus, this common bone disease mostly affects women. Female lupus patients may have nearly five times the risk of a fracture from osteoporosis, according to the U.S. National Institutes of Health (NIH). Bone loss can result from corticosteroids used to treat lupus and from inactivity due to pain and fatigue caused by lupus.

• Pregnancy difficulties. Miscarriages are sometimes the first sign of SLE. Babies born to SLE patients may have heart, liver or skin conditions. A nationwide survey of more than 18 million American women found that SLE patients were more likely than other pregnant women to have a pregnancy-related death, require a Caesarean section or develop complications such as deep vein thrombosis (blood clot in a leg), sepsis (serious blood infection) or anemia (insufficient number of red blood cells). With proper medical care, however, most women with lupus can have a normal pregnancy and healthy baby, according to the NIH.

The American College of Rheumatology (ACR) suggests that SLE patients of childbearing age use birth control during flares and try to time pregnancies during periods of long remission. Recent studies indicate that SLE patients can safely take oral contraceptives, which were once thought to worsen the disease.

• Kidney damage. Inflammation of the kidneys can impair the ability to get rid of the waste products from the body effectively. Black patients are particularly prone to renal damage from lupus, researchers have found. Treating kidney disease early reduces the risk of chronic renal failure, which requires lifelong dialysis or a kidney transplant. Patients should ask their physician about regularly having a special urine test - not a routine urinalysis - for microalbuminuria (small amounts of protein in the urine), an early sign of kidney disease.
  
  
• Cardiovascular diseases. SLE damages the blood vessels, accelerates atherosclerosis (hardening and narrowing of the arteries) and can lead to stroke, heart attack or heart failure. A stroke is sometimes a symptom of SLE in an undiagnosed individual.
  
  
• Chest pain. Patients may suffer from pleurisy (inflammation of the membrane covering the lungs) or pericarditis (inflammation of the membrane covering the heart).
  
  
• Damage to the nervous system. SLE can cause a wide range of neurological disorders, including headaches, vision problems, seizures and mental disorders.

However, with regular physician visits and medication, most lupus patients do not develop serious health problems or have a shortened lifespan, according to the U.S. National Institute of Neurological Disorders and Stroke.

There are four main forms of lupus:

• Systemic lupus erythematosus. The most common and most serious form of this autoimmune disease. SLE can damage many parts of the body, including the joints, skin, kidneys, blood, blood vessels, heart, nerves and brain. SLE is chronic, lasting the rest of a person's life, typically with long periods of good health broken up by exacerbations (flares) of the disease. In many patients the disease is mild, with symptoms such as skin rashes and joint pain, but in others there are serious and sometimes deadly complications such as stroke, organ failure or pregnancy complications.
  
  
• Drug-induced lupus. A condition brought on by longtime use of certain medications. The Lupus Foundation of America states that at least 38 medications are known to cause drug-induced lupus, notably procainamide and quinidine (both for heart rhythm abnormalities) and hydralazine, a vasodilator prescribed for high blood pressure. There is little risk of developing lupus after using other medications, according to the organization. Drug-induced lupus generally goes away when the medication is discontinued.
  
  
• Discoid (cutaneous) lupus. A chronic condition that generally affects only the skin. It is typically characterized by a red rash, skin that changes color and sores, particularly on the face or scalp. Some patients are especially prone to sores on skin exposed to the sun. Patients may experience permanent scarring and hair loss. About 10 percent of lupus patients have the discoid form, according to the American College of Rheumatology. The Lupus Foundation of America estimates that 10 percent of patients who appear to have discoid lupus will go on to develop SLE and may in fact have had that form all along.
  
  
• Neonatal lupus. A rare disease that affects newborns, resulting from autoantibodies received from the mother's bloodstream. A rash affects the infant within a few weeks of birth and may last up to six months, and rarely the infant's heart is affected, according to the Lupus Foundation of America.

Longtime use of certain medications can cause drug-induced lupus, but scientists do not know what causes other types of lupus. Many suspect that genes and hormones predispose an individual to the disease, which may be triggered by certain environmental factors. Factors that influence a person's chances of developing lupus include:

• Sex and age. Ninety percent of lupus patients are women, usually in their childbearing years of 15 to 45, supporting the theory that female hormones play a role in role in the disease. However, early results from the landmark Carolina Lupus Study have unexpectedly not linked greater exposure to estrogen to increased risk of systemic lupus erythematosus (SLE), the most common and serious form of lupus.
  
  
• Race. Lupus is two to three times more common in people of color, according to the Lupus Foundation of America. The disease is typically more severe in blacks and Asians, according to the American College of Rheumatology (ACR).
  
  
• Genetics. Scientists have identified several genes as risk factors for lupus. Genes are believed to account for at least part of the racial disparity in prevalence of SLE. Researchers have identified a cluster of 14 genes, known as the interferon expression signature, in some SLE patients who develop severe complications such as kidney failure.
  
  
• Family history. About 5 percent of children develop lupus if a parent had it, and 20 percent of patients have a parent or sibling who has or may develop lupus, according to the Lupus Foundation of America.

There is much speculation about the possible environmental triggers of lupus. Workers exposed to crystalline silica (quartz) dust, a mineral found in rocks and soil, may have 2 to 4 times the risk of developing the disease, the National Institutes of Health reported in 2007, based on early results from the Carolina Lupus Study. Viruses, sunlight and stress have been implicated but not proven. According to the U.S. Food and Drug Administration (FDA), triggers in susceptible people may include:

• Infections such as the Epstein-Barr virus
• Ultraviolet light
• Medications such as sulfa antibiotics
• The herbal supplement echinacea (purple coneflower), which many people take in an effort to prevent or relieve colds

Indicators of lupus vary widely in occurrence and severity among patients. A butterfly-shaped rash over the nose and cheeks is a common sign. However, a butterfly rash can also be caused by an inflammatory skin and muscle disease called dermatomyositis.

Other signs and symptoms of lupus, which can also suggest many other conditions, may include:

• Red rashes or changes in skin color
  
  
• Sensitivity to the sun (photosensitivity)
  
  
• Skin, nasal or mouth sores
  
  
• Arthritis-like joint pain or swelling (edema)
  
  
• Muscle pain
  
  
• Fatigue
  
  
• Unexplained fever
  
  
• Hair loss
  
  
• Chest pain, especially when breathing in deeply, and shortness of breath (caused by inflammation of membranes covering the lungs or heart)
  
  
• Kidney problems, which may cause hematuria (blood in the urine) or proteinuria (protein in the urine, detectable in a urine test) or be marked by abdominal, pelvic or back pain
  
  
• Pale or purple fingers or toes due to impaired circulation, called Raynaud's phenomenon
  
  
• Swollen lymph nodes and sometimes an enlarged spleen (splenomegaly)
  
  
• Swelling around the eyes or in the legs
  
  
• Blood clots
  
  
• Nausea, vomiting, diarrhea or unexplained weight loss
  
  
• Miscarriages

Less commonly, according to the U.S. government's National Institute of Arthritis and Musculoskeletal and Skin Diseases, people with systemic lupus erythematosus (SLE) may experience:

• Headaches
• Dizziness
• Anemia (insufficient number of red blood cells)
• Depression
• Confusion
• Seizures

Signs of SLE may also include epilepsy, personality changes, psychosis, neuropathy (nerve damage), paralysis or stroke, according to the National Institute of Neurological Disorders and Stroke.

Diagnosis of lupus, especially systemic lupus erythematosus (SLE), is difficult and sometimes takes years. There is no single definitive test for SLE. Symptoms vary widely among patients and can suggest many other conditions.

SLE is sometimes called "the great impostor" because it can be mistaken for many other diseases, such as rheumatoid arthritis, polymyalgia rheumatica, chronic fatigue syndrome, fibromyalgia, multiple sclerosis or Sjogren's syndrome. A physician may need to test for and rule out other conditions.

An accurate and complete medical history is crucial in diagnosing and monitoring lupus. The U.S. National Institutes of Health (NIH) suggests that individuals keep a checklist of symptoms (e.g., joint pain, rash) and bring it to a physician appointment. The list should include types of symptoms, sites where they occur, when they were first noticed, frequency of occurrence and recent dates.

The physician will likely conduct a physical examination with a focus on the joints, skin, muscles and other areas affected by pain, rashes or other symptoms. Diagnostic tests may include:

• Blood tests. The ANA test detects antinuclear antibodies (immune cells that attacks foreign substances), which, according to the American College of Rheumatology, are present in virtually everyone who has lupus. This test is not definitive for lupus, however, because antinuclear antibodies can also be present with other immune diseases such as rheumatoid arthritis, polymyositis or scleroderma. Also, 25 percent of the population may be ANA positive but never develop lupus.

More specific tests, including the anti-double strand DNA (dsDNA) and anti-smith antibodies (Sm), can help confirm a diagnosis. A number of other immune system blood tests may also help in diagnosis, especially in patients with a negative ANA test.

Other blood tests used in diagnosing lupus may include:

• • Complement. Can help suggest lupus by indicating depressed levels of proteins that attack foreign substances. Scientists have linked abnormal levels of certain complement proteins on red blood cells (e.g., complement 4, complement receptor 1) to lupus.
    
    
  • Complete blood count. Detects low red blood cell count (anemia), low white blood cell count or low platelet count that may be due to lupus.
    
    
  • Blood differential. Measures the number of white blood cells, which may be altered because of lupus or other diseases.
    
    
  • Sedimentation rate or or C-reactive protein test. Nonspecific indicators of inflammation.
    
    
  • Rheumatoid factor test. Measures an antibody called rheumatoid factor that can indicate lupus, rheumatoid arthritis or other autoimmune diseases.
    
    
  • Waste product test. Can reveal kidney damage that could be due to SLE.
    
    
• Urine tests. Urinalysis may reveal protein in the urine or evidence of kidney inflammation, both of which can result from SLE. A special microalbuminuria test is needed to find the small amounts of protein that are an early sign of kidney damage. Blood in urine (hematuria) may indicate kidney damage caused by SLE or other disorders. A creatinine urine test can also reveal such kidney damage.
  
  
• Biopsy. Skin tissue samples examined under a microscope can help diagnose discoid (cutaneous) lupus, and a skin or kidney biopsy may reveal tissue damage characteristic of SLE.
  
  
• Imaging tests. Techniques such as MRI, CAT scan, ultrasound, radionuclide imaging or arthrography may be used to detect organ and tissue damage that may be due to lupus.

A primary care physician who suspects lupus may refer a patient to a rheumatologist (specialist in diseases of the joints, muscles and bones) or specialist in autoimmune disorders (immunologist) for additional expertise in diagnosis and treatment. Patients with discoid (cutaneous) lupus may be referred to a dermatologist.

Drug-inducted lupus can be resolved by discontinuing the medication under a physician's supervision, but there is no known cure for other forms of lupus including systemic lupus erythematosus (SLE). Treatment focuses on relieving symptoms, preventing relapses (flares) and avoiding complications such as organ damage. Most patients are able to lead long, fairly normal lives with extended periods of remission disrupted by occasional flares. It is important for patients to receive regular medical care even during remissions.

There are many medications that can help relieve SLE and other forms of lupus. Typically a physician will try milder drugs and use stronger doses or medications if necessary. Medications may include:

• Corticosteroids for inflammation. These may be given by mouth, by injection or in topical creams. Risks of long-term use of corticosteroids include osteoporosis, fractures, diabetes, glaucoma and cataracts.

• Aspirin or other NSAIDs (nonsteroidal anti-inflammatory drugs) for inflammation, fever and pain.
  
  
• Topical creams for rashes.
  
  
• Anti-malaria drugs and chemotherapy drugs. Though originally developed to treat other diseases, these medications can relieve symptoms of SLE.
  
  
• Intravenous immunoglobin (IVIG). This blood product may be used as an anti-inflammatory.
  
  
• DMARDs (disease-modifying antirheumatic drugs). These medications can decrease joint damage, reduce swelling and pain and sometimes slow or halt progression of the disease. Potential side effects include increased risk of infection and gastrointestinal, liver or kidney complications.
  
  
• Other immunosuppressives. Patients with damage to kidneys or other organs may be prescribed drugs that suppress the immune system, including biologic response modifiers (BRMs) such as TNF inhibitors. Potential risks of such medications include infections.

These medications can have strong side effects, but the physician may be able to reduce or discontinue use of the drugs during remissions. Patients should inform their physician of side effects and not discontinue medications on their own. The U.S. National Institutes of Health (NIH) recommends that patients taking corticosteroids or anti-malaria drugs have an annual eye examination to check for eye problems that may result. Patients using corticosteroids long-term are advised to ask about osteoporosis monitoring tests such as a DEXA scan, a type of x-ray.

Clinical trials are being conducted to assess new medications for lupus. Interested patients can ask their physician for details or check the web site of the NIH (www.clinicaltrials.gov).

There is no research showing that alternative treatments (those not part of standard medical care) benefit lupus patients, according to the NIH. Patients should discuss any alternative treatments with their physician before trying them. Herbal supplements and vitamins interact with some medications, and some supplements may contain unsafe ingredients.

Lupus patients often are treated by a rheumatologist or an immunologist (physician who specializes in immune disorders). Other health professionals can also help:

• Specialist physicians. Dermatologists treat the skin conditions, orthopedists the bone and joint complications, nephrologists the kidney disorders, neurologists the nerve disorders, pulmonologists the lung disorders and cardiologists the cardiovascular problems that may result from SLE.
  
  
• Psychologists or psychiatrists. Address depression, anxiety, stress, self-esteem, altered body image and other issues that often arise.
  
  
• Occupational therapists. Offer instruction in energy conservation, work modification, task simplification, assistive devices, posture and ergonomics, relaxation techniques, stress management, and other ways to adapt and avoid fatigue.
  
  
• Physical therapists. Offer exercises to maintain range of motion (ROM) and reduce stiffness, and modalities such as therapeutic ultrasound and thermotherapy to reduce pain and maintain ROM.
  
  
• Dietitians. Can recommend beneficial foods, such as those rich in vitamin K to ease skin conditions and those rich in calcium and vitamin D to strengthen bones.
  
  
• Orthopedic surgeons. Joint surgery is usually the last treatment option for damaged joints.

There is no known way to prevent lupus, but patients can take action to help avoid flares and complications. These steps include:

• Watching for warning signs. Many people with SLE experience symptoms before a flare strikes. These may include fatigue, pain, rashes, fever, abdominal pain, headache or dizziness.
  
  
• Rest. It is important for individuals to conserve energy if a flare is imminent. Setting priorities and delegating tasks to others are essential at times.
  
  
• Limiting exposure to ultraviolet light. Sunlight, tanning beds and other sources of ultraviolet rays can cause lupus to flare. Patients may need supplements of vitamin D, which the skin makes with the help of solar radiation. Some individuals are even sensitive to indoor light and may need dimmer lighting to prevent or relieve flares.
  
  
• Stress management. Meditation, yoga, biofeedback and other techniques often help individuals cope. Some patients find relief in lupus support groups.
  
  
• Exercise. Aerobic, ROM and strengthening exercises, particularly during remissions, can help people endure flares. Patients should get approval from their physician before starting an exercise program. ROM activities may be beneficial during flares, but other exercises may need to be restricted.
  
  
• Nutrition. A healthy diet helps the body cope with flares. The NIH recommends a diet rich in calcium and vitamin D to promote bone health and lower the risk of SLE-related osteoporosis and fractures.

Because SLE raises the risk of atherosclerosis and cardiovascular disease, patients should take preventive measures such as controlling blood pressure and cholesterol and not smoking, according to the American College of Rheumatology. Patients should also be screened regularly for kidney disease and have it treated promptly and aggressively.

Patients at increased risk for blood clots (e.g., blood testing reveals antiphospholipid antibodies) should not take estrogen, the American College of Rheumatology recommends.

Lupus may affect many aspects of a person's life, but being proactive can empower individuals physically and psychologically.

Preparing questions in advance can help patients have more meaningful discussions with their physicians regarding their conditions. Patients may wish to ask their doctor the following questions about lupus:

1. Could my joint pain, rashes or other symptoms be due to lupus? What other conditions could be causing my symptoms?
   
   
2. How is lupus diagnosed? What tests do you recommend for me? What do these tests involve?
   
   
3. What do my test results show? What form of lupus do I have?
   
   
4. Do I also have overlap symptoms of other conditions such as rheumatoid arthritis or scleroderma?
   
   
5. What might have caused my lupus?
   
   
6. What is the expected course of my case of case of lupus? Is it temporary or lifelong? Is it likely to come and go, remain steady, improve or worsen?
   
   
7. What are my treatment options? Which do you recommend?
   
   
8. At what point should I notify you if my medication is causing side effects? Does my medication require that I have screening tests of my vision or anything else?
   
   
9. Do I need to see any specialists?
   
   
10. How can I prevent or control flares?
    
    
11. What exercises do you recommend? How can I tell when I should exercise and when I should rest?
    
    
12. Should I make any adjustments in my diet? Should I see a dietitian? Do I need supplements of vitamin D, calcium or anything else?
    
    
13. Do I need to avoid the sun or strong artificial light?
    
    
14. Do I need regular medical appointments even when I'm in remission and feeling fine?
    
    
15. Do you recommend occupational therapy, physical therapy, stress management techniques, support groups or anything else to help me cope?
    
    
16. Should I be monitored for osteoporosis with a DEXA scan or other test? How often?
    
    
17. Is it safe for me to use birth control pills or take estrogen?
    
    
18. Can I have a safe pregnancy with SLE? Is it important for me to time my pregnancy during a long period of remission?
    
    
19. If my spouse or I have lupus, what are the chances that our baby will inherit it?
    
    
20. Is lupus likely to run in my family? Should relatives undergo any testing or monitoring? Can it be prevented?
    
    
21. Can any of my medications cause drug-induced lupus or worsen my systemic lupus?