LUPUS CAUSES AND TREATMENT

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, brain, heart and lungs.

Lupus can be difficult to diagnose because its signs and symptoms often mimic those of other ailments. The most distinctive sign of lupus — a facial rash that resembles the wings of a butterfly unfolding across both cheeks — occurs in many but not all cases of lupus.

Some people are born with a tendency toward developing lupus, which may be triggered by infections, certain drugs or even sunlight. While there's no cure for lupus, treatments can help control symptoms.

SYMPTOMS


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. The most common signs and symptoms include:

Fatigue and fever
Joint pain, stiffness and swelling
Butterfly-shaped rash on the face that covers the cheeks and bridge of the nose
Skin lesions that appear or worsen with sun exposure
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
Headaches, confusion, memory loss
When to see a doctor

See your doctor if you develop an unexplained rash, ongoing fever, persistent aching or fatigue.

CAUSES

Lupus occurs when your immune system attacks healthy tissue in your body. It's likely that lupus results from a combination of your genetics and your environment. It appears that people with an inherited predisposition for lupus may develop the disease when they come into contact with something in the environment that can trigger lupus. The cause for lupus in most cases, however, is unknown. Some potential triggers include:

Sunlight. Exposure to the sun may bring on lupus skin lesions or trigger an internal response in susceptible people.
Medications. Lupus can be triggered by certain types of anti-seizure medications, blood pressure medications and antibiotics. People who have drug-induced lupus usually see their symptoms go away when they stop taking the medication.

RISK FACTORS

Factors that may increase your risk of lupus include:

Your sex. Lupus is more common in women.
Age. Although lupus affects people of all ages, it's most often diagnosed between the ages of 15 and 40.
Race. Lupus is more common in African Americans, Hispanics and Asians.

COMPLICATIONS

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. Signs and symptoms of kidney problems may include generalized itching, chest pain, nausea, vomiting and leg swelling (edema).
Brain. If your brain is affected by lupus, you may experience headaches, dizziness, behavior changes, hallucinations, and even strokes or seizures. Many people with lupus experience memory problems and may have 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).
Lungs. Having lupus increases your chances of developing an inflammation of the chest cavity lining (pleurisy), which can make breathing painful.
Heart. Lupus can cause inflammation of your heart muscle, your arteries or heart membrane (pericarditis). The risk of cardiovascular disease and heart attacks increases greatly as well.
Other types of complications

Having lupus also increase your risk of:

Infection. People with lupus are more vulnerable to infection because both the disease and its treatments weaken the immune system. Infections that most commonly affect people with lupus include urinary tract infections, respiratory infections, yeast infections, salmonella, herpes and shingles.
Cancer. Having lupus appears to increase your risk of cancer.
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 most commonly affected.
Pregnancy complications. Women with lupus have an increased risk of miscarriage. Lupus increases the risk of high blood pressure during pregnancy (preeclampsia) and preterm birth. To reduce the risk of these complications, doctors recommend delaying pregnancy until your disease has been under control for at least 6 months.

PREPARING FOR YOUR APPOINTMENT

You're likely to start by seeing your family doctor or primary care provider, but he or she may refer you to specialist in the diagnosis and treatment of arthritis and other inflammatory joint conditions (rheumatologist). 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.

What you can do

Before your appointment, you may want to write a list of answers to the following questions:

When did your symptoms begin? Do they come and go?
Does anything seem to trigger your symptoms?
Have your parents or siblings had lupus or other autoimmune disorders?
What medications and supplements do you take regularly?
You may also want to write down questions to ask your doctor, such as:

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 doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may leave time to go over any points you want to spend more time on. Your doctor may ask:

Does sun exposure cause you to develop skin rashes?
Do your fingers become pale, numb or uncomfortable in the cold?
Do your symptoms include any problems with memory or concentration?
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 pregnant or do you plan to become pregnant?

TEST AND DIAGNOSIS

Diagnosing lupus is difficult because signs and symptoms vary considerably from person to person. Signs and symptoms of lupus may vary over time and overlap with those of many other disorders. No one test can diagnose lupus. The combination of blood and urine tests, signs and symptoms, and physical examination findings leads to the diagnosis.

Laboratory tests

Blood and urine tests may include:

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. It may be elevated if you have lupus, another inflammatory condition, cancer 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. While most people with lupus have a positive ANA test, most people with a positive ANA do not have lupus. If you test positive for ANA, your doctor may advise more-specific antibody testing.
Imaging tests

If your doctor suspects that lupus is affecting your lungs or heart, he or she may suggest:

Chest X-ray. An image of your chest may reveal abnormal shadows that suggest fluid or inflammation in your lungs.
Echocardiogram. This test uses sound waves to produce real-time images of your beating heart. It can check for problems with your valves and other portions of your heart.
Biopsy

Lupus can harm your kidneys in many different ways and treatments can vary, depending on the type of damage that occurs. In some cases, it's necessary to test a small sample of kidney tissue to determine what the best treatment might be. The sample can be obtained with a needle, or through a small incision.

TREATMENT

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. The medications most commonly used to control lupus include:

Nonsteroidal anti-inflammatory drugs (NSAIDs). Over-the-counter NSAIDs, such as naproxen (Aleve) and ibuprofen (Advil, Motrin, others), may be used to treat pain, swelling and fever associated with lupus. Stronger NSAIDs are available by prescription. Side effects of NSAIDs include stomach bleeding, kidney problems and an increased risk of heart problems.
Antimalarial drugs. Medications commonly used to treat malaria, such as hydroxychloroquine (Plaquenil), also can help control lupus. Side effects can include stomach upset and, very rarely, damage to the retina of the eye.
Corticosteroids. Prednisone and other types of corticosteroids can counter the inflammation of lupus, but often produce 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.
Immune suppressants. Drugs that suppress the immune system may be helpful in serious cases of lupus. Examples include cyclophosphamide (Cytoxan), azathioprine (Imuran, Azasan), mycophenolate (Cellcept), leflunomide (Arava) and methotrexate (Trexall). Potential side effects may include an increased risk of infection, liver damage, decreased fertility and an increased risk of cancer. A newer medication, belimumab (Benlysta) also reduces lupus symptoms in some people. Side effects include nausea, diarrhea and fever.

HOME REMEDIES

Take steps to care for your body if you have lupus. Simple measures 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.
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 55 every time you go outside.
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.
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.

ALTERNATIVE MEDICINE

Sometimes alternative or complementary medicine may benefit people with lupus. However, these therapies are usually used with conventional medications. Discuss these treatments with your doctor before initiating them on your own. He or she can help you weigh the benefits and risks and tell you if the treatments will interfere with your current lupus medications.

Complementary and alternative treatments for lupus include:

Dehydroepiandrosterone (DHEA). Supplements containing this hormone have been shown to reduce the dose of steroids needed to stabilize symptoms in some people who have lupus.
Flaxseed. Flaxseed contains a fatty acid called alpha-linolenic acid, which may decrease inflammation in the body. Some studies have found that flaxseed may improve kidney function in people who have lupus that affects the kidneys. Side effects of flaxseed include bloating and abdominal pain.
Fish oil. Fish oil supplements contain omega-3 fatty acids that may be beneficial for people with lupus. Preliminary studies have found some promise, though more study is needed. Side effects of fish oil supplements can include nausea, belching and a fishy taste in the mouth.
Vitamin D. There is some evidence to suggest that people with lupus may benefit from supplemental vitamin D.

COPING AND SUPPORT

If you have lupus, you're likely to have a range of painful feelings about your condition, from fear to extreme frustration. The challenges of living with lupus increase your risk of depression and related mental health problems, such as anxiety, stress and low self-esteem. To help you cope with lupus, try to:

Learn all you can about lupus. Write down all the questions you have about lupus and ask them at your next appointment. Ask your doctor or nurse for reputable sources of further information. The more you know about lupus, the more confident you'll feel in your treatment choices.
Gather support among your friends and family. Talk about lupus with your friends and family and explain ways they can help out when you're having flares. Lupus can be frustrating for your loved ones because they usually can't see it and you may not appear sick. They can't tell if you're having a good day or a bad day unless you tell them. Be open about what you're feeling so that your friends and family know what to expect.
Take time for yourself. Cope with stress in your life by taking time for yourself. Use that time to read, meditate, listen to music or write in a journal. Find activities that calm and renew you.
Connect with others who have lupus. Talk to other people who have lupus. You can connect with other people who have lupus through support groups in your community or through online message boards. Other people with lupus can offer unique support because they're facing many of the same obstacles and frustrations that you're facing.

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Lupus is an autoimmune disease where the body's immune system becomes hyperactive and attacks normal, healthy tissue. This results in symptoms such as inflammation, swelling, and damage to joints, skin, kidneys, blood, the heart, and lungs.

Under normal function, the immune system makes proteins called antibodies in order to protect and fight against antigens such as viruses and bacteria.

Lupus makes the immune system unable to differentiate between antigens (a substance capable of inducing a specific immune response) and healthy tissue. This leads the immune system to direct antibodies against the healthy tissue - not just antigens - causing swelling, pain, and tissue damage.

Here are some key points about lupus. More detail and supporting information is in the main article.
Lupus is an autoimmune disease - it is caused by problems in the body's immune system.
The type that we refer to simply as lupus is known as systemic lupus erythematosus or SLE.
Other types of lupus include discoid (cutaneous), drug-induced, and neonatal.
According to the Lupus Foundation of America, 1.5 to 2 million Americans have some form of lupus.
It is also said that 5 million people worldwide suffer from some form of Lupus.
Women are diagnosed 9 times more often than men and usually between the ages of 15 and 45.
Risk factors include exposure to sunlight, certain prescription medications, infection with Epstein-Barr virus, and exposure to certain chemicals.
Most Doctors believe that lupus results from both genetic and environmental stimuli.
Environmental factors include extreme stress, exposure to ultraviolet light, smoking, some medications and antibiotics, infections and the Epstein-Barr virus (in children).
Symptoms of lupus include achy joints, swelling of hands and feet, fever, fatigue, skin lesions, rash, anemia, chest pain, light sensitivity, hair loss and more.
Treatments for Lupus include corticosteroids, immunosuppressive drugs and lifestyle changes.
There is currently no cure for lupus.

Several different kinds of lupus have been identified, but the type that we refer to simply as lupus is known as systemic lupus erythematosus or SLE. Other types include discoid (cutaneous), drug-induced, and neonatal.
Patients with discoid lupus have a version of the disease that is limited to the skin. It is characterized by a rash that appears on the face, neck, and scalp, and it does not affect internal organs. Less than 10% of patients with discoid lupus progress into the systemic form of the disease, but there is no way to predict or prevent the path of the disease.

SLE is more severe than discoid lupus because it can affect any of the body's organs or organ systems. Some people may present inflammation or other problems with only skin and joints, while other SLE sufferers will see joints, lungs, kidneys, blood, and/or the heart affected. This type of lupus is also often characterized by periods of flare (when the disease is active) and periods of remission (when the disease is dormant).

Drug-induced lupus is caused by a reaction with certain prescription drugs and causes symptoms very similar to SLE. The drugs most commonly associated with this form of lupus are a hypertension medication called hydralazine and a heart arrhythmia medication called procainamide, but there are some 400 other drugs that can also cause the condition. Drug-induced lupus is known to subside after the patient stops taking the triggering medication.
A rare condition, neonatal lupus occurs when a mother passes autoantibodies to a fetus. The unborn and newborn child can have skin rashes and other complications with the heart and blood. Usually a rash appears but eventually fades within the first six months of the child's life.

Who is affected by lupus?
According to the Lupus Foundation of America (LFA), 1.5 to 2 million Americans have some form of lupus. The prevalence is about 40 cases per 100,000 persons among Northern Europeans and 200 per 100,000 persons among African-Americans. Although the disease affects both males and females, women are diagnosed 9 times more often than men, usually between the ages of 15 and 45. African-American women suffer from more severe symptoms and a higher mortality rate.

Other risk factors include exposure to sunlight, certain prescription medications, infection with Epstein-Barr virus, and exposure to certain chemicals.

What causes lupus?Photograph of healthcare professionals
Although doctors are do not know exactly what causes lupus and other autoimmune diseases, most believe that lupus results from both genetic and environmental stimuli.

Since lupus is known to occur within families, doctors believe that it is possible to inherit a genetic predisposition to lupus. There are no known genes, however, that directly cause the illness. It is probable that having an inherited predisposition for lupus makes the disease more likely only after coming into contact with some environmental trigger.

The higher number of lupus cases in females than in males may indicate that the disease can be triggered by certain hormones.

Physicians believe that hormones such as estrogen regulate the progression of the disease because symptoms tend to flare before menstrual periods and/or during pregnancy.

Certain environmental factors have been known to cause lupus symptoms. These include:

Extreme stress
Exposure to ultraviolet light, usually from sunlight
Smoking
Some medications and antibiotics, especially those in the sulfa and penicillin groups
Some infections, such as cytomegalovirus (CMV), parvovirus (such as fifth disease), hepatitis C infections, and the Epstein-Barr virus (in children)
Chemical exposure to compounds such as trichloroethylene in well water and dust
What are the symptoms of lupus?
Since no two cases of lupus are exactly alike, there is a wide range of symptoms that are known to affect many parts of the body. Sometimes symptoms develop slowly or appear suddenly; they can be mild, severe, temporary, or permanent. Most people with lupus experience symptoms in only a few organs, but more serious cases can lead to problems with kidneys, the heart, the lungs, blood, or the nervous system.

Lupus episodes, or flares, are usually noted by a worsening of some of the following symptoms:

The symptoms of Lupus
Achy joints (arthralgia), arthritis, and swollen joints, especially in wrists, small joints of the hands, elbows, knees, and ankles
Swelling of the hands and feet due to kidney problems
Fever of more than 100 degrees F (38 degrees C)
Prolonged or extreme fatigue
Skin lesions or rashes, especially on the arms, hands, face, neck, or back
Butterfly-shaped rash (malar rash) across the cheeks and nose
Anemia (oxygen carrying deficiency of red blood cells)
Pain in the chest on deep breathing or shortness of breath
Sun or light sensitivity (photosensitivity)
Hair loss or alopecia
Abnormal blood clotting problems
Raynaud's phenomenon: fingers turn white and/or blue or red in the cold
Seizures
Mouth or nose ulcers
Weight loss or gain
Dry eyes
Easy bruising
Anxiety, depression, headaches, and memory loss
Lupus can also lead to complications in several areas of the body. These include:

Kidneys - serious kidney damage is a primary cause of death for lupus sufferers.
Central nervous system - lupus can cause headaches, dizziness, memory problems, seizures, and behavioral changes.
Blood and vessels - lupus causes an increased risk of anemia, bleeding, blood clotting, and vessel inflammation
Lungs - noninfectious pneumonia and difficulty breathing due to inflammation of the chest cavity are more likely with lupus
Heart - heart muscle and artery inflammation are more likely with the disease, and lupus increases the chances of cardiovascular disease and heart attacks.
Infection - lupus treatments tend to depress the immune system making your body more vulnerable to infection.
Cancer - lupus increases the risk of cancer, especially of non-Hodgkin's lymphoma, lung cancer, and liver cancer
Bone tissue death - a lower blood supply to bone tissue leads to tiny breaks and eventual death of bone. This is most common in the hip bone.
Pregnancy - lupus increases the risk of miscarriage, hypertension during pregnancy, and preterm birth.

How is lupus diagnosed?
As signs and symptoms vary considerably from person to person, there is no single diagnostic test that can confirm lupus. In addition, signs and symptoms tend to change over time and are similar to those of other disorders and diseases. These fluctuations in disease activity make lupus extremely challenging to diagnose.

Currently, doctors use guidelines established by The American College of Rheumatology (ACR) to diagnose lupus (SLE). The guidelines focus on eleven abnormalities that, when combined, suggest that the patient has lupus. To be classified as having SLE, a patient must meet 4 of the following 11 symptoms at any time since the onset of the disease:

Serositis - inflammation of the membrane around the lungs (pleuritis) or the heart (pericarditis)
Mucosal ulcers - small sores found in the lining of the mouth and nose
Arthritis - nonerosive arthritis (tenderness, swelling, pain) of two or more peripheral joints
Photosensitivity - skin rash or other symptoms caused by exposure to ultraviolet light
Blood disorder - hemolytic anemia (low red blood cell count), leucopenia and lymphopenia (low white blood cell count), or thrombocytopenia (low platelet count)
Renal (kidney) disorder - high protein count in urine
Antinuclear antibody test positive
Immunologic disorder - positives on anti-Smith, anti-ds DNA, antiphospholipid antibody tests.
Neurologic disorder - seizures or psychosis
Malar rash - rash on cheeks
Discoid rash - red, scaly patches on skin that cause scarring
In addition to the above tests, doctors will often conduct a variety of blood tests such as:

Complete blood count (CBC) to detect anemia, low platelet count, and low white blood cell count
Erythrocyte sedimentation rate (ESR) to determine the rate at which red blood cells settle to the bottom of a tube in an hour. Rates faster than normal may indicate lupus or another systemic disease, inflammatory condition, or infection.
Kidney and liver assessment to look for certain enzymes and albumin
Urinalysis to measure protein levels or red blood cells in the urine
Syphilis test to determine if anti-phospholipid antibodies are in the blood.
How is lupus treated?Photograph of Doctor making notes
There is currently no cure for lupus, nor has there been a new drug to treat the disease in the last 50 years, although there are a number of new drugs currently being researched or in clinical trials. However, early diagnosis and proper medical treatment can significantly help control the disease and its symptoms. Treating lupus effectively consists of minimizing symptoms, reducing inflammation and pain, helping maintain normal function, and preventing serious complications.

Since the disease affects each person differently, treatments are usually tailored to the specific problems that arise in each person. Medications and dosages will also vary depending on the severity of the disease.