What is Lupus?

Lupus is a chronic, autoimmune disease, which causes inflammation of various parts of the body, especially the skin, joints, blood and kidneys. The body's immune system normally makes proteins called antibodies to protect the body against viruses, bacteria and other foreign materials. These foreign materials are called antigens. In an autoimmune disorder such as lupus, the immune system loses its ability to tell the difference between foreign substances (antigens) and its own cells and tissues. The immune system then makes antibodies directed against itself. These antibodies, called auto-antibodies, react with the "self" antigens to form immune complexes. The immune complexes build up in the tissues and can cause inflammation, injury to tissue, and pain.

TYPES OF Lupus

There are three types of lupus. Cutaneous Lupus (also known as DISCOID Lupus) is limited to the skin and is identified by a rash that may appear on the face, neck, or scalp. The rash may be more apparent on areas of the skin exposed to ultraviolet light (e.g., sunlight, fluorescent light). Although there are many types of lupus rash, the most common is raised, scaly and red, but not itchy. Systemic Lupus Erythematosus (SLE) is usually more severe than DISCOID Lupus. This type of lupus can cause inflammation in a number of organs. For some people this may be limited to skin and joint involvement. In others, the joints, lungs, kidneys, blood or other organs and/or tissues may be affected. SLE may include periods in which few, if any, symptoms are evident (remission) and other times in which the disease becomes active (flare). Certain prescribed drugs can create a lupus-like syndrome (called drug-induced lupus), which is similar to SLE. This type of lupus very rarely affects either the kidneys or the nervous system. The drugs most commonly connected with drug-induced lupus are hydrolyzine (used to treat hypertension) procainamide (used to treat irregular heart rhythms) and quinidine (also used to treat irregular heart rhythms). Not everyone who takes these drugs will develop drug-induced lupus. When the medications are discontinued, the symptoms of lupus usually fade.

CAUSE

Although the causes of lupus remain unknown, both genetic and environmental factors may play a role in its development. While an individual's genetic structure may increase the chance of developing lupus, it probably takes some kind of environmental factor to trigger the illness. Two known factors are ultraviolet rays (which can cause sunburns) and certain drugs. Infections and surgery may also trigger the symptoms of lupus. Since lupus occurs ten times more frequently in women than in men, researchers believe that hormonal factors may also influence the development of the disease. Statistics show that only about 5% of children born to individuals with lupus will develop the illness. This means that most children will not develop the disease even if one of the parents has lupus. Only one in ten individuals will have a close relative (parent, sibling or child) who already has or may develop lupus.

SYMPTOMS OF Lupus

Although lupus can affect any part of the body, most people with lupus experience symptoms in only a few organs. The table below lists the most common symptoms of lupus.

Achy joints
Fever over 100 degrees F (38 degree C)
Swollen joints (arthritis)
Skin rashes
Anemia
Kidney involvement
Pain in the chest on deep breaths in an unrelated position or movement (pleurisy)
Butterfly-shaped rash across the cheeks and nose
Photosensitivity
Hair loss
Fingers turning white and/or blue in the cold (Raynauds Phenomenon)
Seizures
Mouth ulcers

Most people with lupus do not experience all symptoms at any one time. The list only serves to alert people to clues that might indicate the presence of lupus in an undiagnosed person, or the increased activity of the disease in a person who has been diagnosed with lupus.

Patients with lupus go through cycles of symptoms, called flares, and periods of well ness, called remission. Other symptoms of lupus include chest pain, hair loss, sensitivity to the sun, pale or, purple fingers and toes from cold and stress, headaches, dizziness, depression, and seizures.

DIAGNOSIS
How does a physician arrive at a diagnosis?

In the early 1980's, physicians in the American Rheumatism Association surveyed large numbers of people with lupus and found eleven symptoms that would help distinguish lupus from other diseases. For a physician to detect lupus, a person must have one of the following symptoms at some time during his or her illness.

Butterfly-shaped rash across the cheek
Discoid rash
Photosensitivity
Mouth or nose ulcers
Arthritis
A positive LE cell test, repeated false blood tests for syphilis (i.e., a blood test incorrectly indicating the presence of syphilis)
Excessive protein in the urine, or cellular cast in the urine
Pericarditis (inflammation of the tissues around the heart) and/or pleurisy
Psychosis and/or convulsions
Hemolytic anemia, low white blood cell count, or low platelet count
Antinuclear antibodies (ANA)

Diagnosing lupus can be difficult. It could take months or even years for doctors to accurately diagnose it.

It is important to provide the doctor with a complete, accurate medical history in order to get an accurate diagnosis.

The medical history, along with a physical examination and laboratory tests, can help the doctor diagnose lupus. Making a diagnosis may take time as new symptoms appear.

No single test can show whether a person has lupus, but several laboratory tests may help diagnose it. Useful tests identify certain blood auto antibodies, often present in people with lupus.

Some tests are used less often but may be helpful if the symptoms’ cause remains un-clear. The doctor may order a biopsy of the skin or kidneys if they are affected. The purpose of a biopsy is to take sample tissue for analysis in the lab.

Some doctors may order a syphilis test because lupus antibodies in the blood may cause the test to be falsely positive. In such a case, a positive test does not mean that a patient has syphilis.

All tests are tools that give the doctor more information for a diagnosis. The doctor will look at the medical history, symptoms, and test results to determine if a person has lu-pus.

Other laboratory tests are used to monitor the progress of lupus once it has been diag-nosed. A complete blood count (CBC), urinalysis, blood chemistries, and erythrocyte sedimentation rate (ESR) tests can provide important information.

Another common test measures the blood level of a group of proteins called com-plements. People with lupus often have low complement levels, especially when lupus flares up.

TREATMENT

Currently, there is no cure for lupus; however, early diagnosis and proper medical treatment can significantly help to control the disease. Symptoms often vary from one individual to another and treatment is based on specific indications in each person. Still, a few general guidelines can be listed:

Regular rest is important when the disease is active. When the disease is in remission, increased physical activity is encouraged to increase joint flexibility and muscle strength.
For the individual who is photosensitive, the regular use of sunscreens will help prevent rashes and irritations. For those who develop rashes, treatment with cortisone creams is very helpful.
Achy joints (arthralgia) and arthritis generally respond to aspirin or aspirin-like drugs (non-steroidal anti-inflammatory drugs).
The anti-malarial drug hydroxcholorquin (Plaquenil) is often prescribed for more severe joint or skin involvement.
Cortisone drugs (the most commonly prescribed is Prednisone) are often used for more severe organ involvement. Not everyone with SLE needs cortisone. Cortisone, particularly in higher doses, has possible hazardous side effects.
If you have a fever (over 100 degrees F), call your doctor.
Go to your doctor for regular checkups. Regular checkups usually include blood and urine tests.
When in doubt, ask. Call a doctor.

Treatment plans should meet the individual patient's needs and may change over time. To develop a treatment plan, the doctor tries to:

Prevent flares
Treat flares when they do occur
Minimize complications

The doctor and patient should reevaluate the plan regularly to ensure that it is as effective as possible.

Several types of drugs are used to treat lupus. For people with joint pain, fever, and swelling, drugs that decrease inflammation, referred to as nonsteroidal anti-inflammatory drugs, or NSAIDs, are often used.

While some NSAIDs are available over the counter, a doctor's prescription is necessary for others.

Common side effects of NSAIDs include stomach upset, heartburn, diarrhea, and fluid retention.

Some lupus patients also develop liver and kidney inflammation while taking NSAIDs. It is especially important to stay in close contact with the doctor while taking NSAIDs.
Antimalarials are another type of drug commonly used to treat lupus. These drugs were originally used to treat malaria, but they are also useful in treating lupus.
Antimalarials may be used alone or in combination with other drugs to treat fatigue, joint pain, skin rashes, and inflammation of the lungs. Continuous treatment with anti-malarials may prevent flares from recurring.

Side effects of antimalarials may include stomach upset and, very rarely, damage to the retina of the eye.

The most common treatment for lupus is corticoid steroid hormones. Corticoid steroids are related to cortisol, a natural anti-inflammatory hormone. They hold back inflammation very quickly.

Corticoid steroids can be given orally, in creams applied to the skin, or by injection. Since they are potent drugs, the doctor will use the lowest dose with the greatest benefit.

Short-term side effects of corticoid steroids include swelling, increased appetite, weight gain, and emotional ups and downs. These side effects usually stop when the drug is stopped.

It can be dangerous to stop taking corticoid steroids suddenly, so it is very important that a doctor recommend changes for the corticoid steroid dose.
Sometimes doctors give very large amounts of corticoid steroid for a short time by vein. With this treatment, typical side effects are less likely and slow withdrawal is not necessary.

Long-term side effects of corticoid steroids can include stretch marks, excessive hair growth, weakened or damaged bones, high blood pressure, damage to the arteries, high blood sugar, infections, and cataracts.

Typically, the higher the dose of corticoid steroids, the more severe the side effects are. The longer corticoid steroids are taken, the greater the risk of side effects becomes.
People with lupus who use corticoid steroids should talk to their doctors about taking supplemental calcium and Vitamin D. These supplements reduce the risk of fragile bones called osteoporosis.
For patients whose kidneys or central nervous systems are affected by lupus, a type of drug called an immunosuppressive may be used. Immunosuppressive hold the immune system back by blocking the production of some immune cells.

Immunosuppressive may be given orally or by IV.

Side effects of immunosuppressive may include nausea, vomiting, hair loss, bladder problems, decreased fertility, and increased risk of cancer and infection. The longer the treatment with immunosuppressive, the higher the risk of side effects becomes.

Since some treatments may cause harmful side effects, it is important to tell the doctor about any side effects right away. It is also important NOT to stop or change treatment without asking the doctor first.

PROGNOSIS

The outlook for people with lupus has significantly improved over the last two decades. Better diagnostic techniques, evaluation methods and a more cautious use of medications have given physicians the tools to more effectively manage lupus symptoms and complications. Twenty years ago, only 40% of the people with lupus were expected to live more than three years following diagnosis. Today, with early diagnosis and current methods of therapy, 80-90% of the people with lupus can look forward to a normal life.

Despite the symptoms of lupus and the possible side effects of treatment, people with lupus CAN have a high quality of life.

One way to manage lupus is to understand the disease and its symptoms. Learning to recognize the warning signs of a flare-up can help the patient take steps to reduce the intensity.

Developing strategies to prevent flare-ups can be helpful. Examples of preventative strategies include limiting exposure to the sun or scheduling adequate rest and quiet times.
It is important for people with lupus to receive regular medical care instead of looking for help only when symptoms get worse. Having a medical exam and tests done on a regular basis allows the doctor to note changes.

People with lupus should receive regular preventive health care, such as gynecological and breast exams.

Regular dental care helps prevent potentially dangerous infections.

If a person is taking corticoid steroids or antimalarial medications, he or she should have a yearly eye exam to screen for and treat eye problems.

For people with lupus, staying healthy takes extra effort and care, so it is especially important to maintain wellness and keep stress low. Some stress reduction measures may include exercise, relaxation, meditation, and prioritizing ways to spend time and energy.

Having a good support system is very important. A support system may include family, friends, medical professionals, community organizations, and organized support groups.

PREVALENCE

More people have lupus than AIDS, cerebral palsy, multiple sclerosis, sickle-cell anemia and cystic fibrosis combined. LFA market research data show that between 1,400,000 and 2,000,000 people were reported to have been diagnosed with lupus. (Study conducted by Buskin/Goldring Research, 1994.)

Lupus affects one out of every 185 Americans. The disease is more prevalent in African Americans, Latinos, Native Americans and Asians. For most people, lupus is a mild disease affecting only a few organs. For others, it may cause serious and even life-threatening problems. Thousands of Americans die each year from lupus-related complications. Although lupus may affect people of either sex and may occur at any age, young females are at greatest risk for the development of the disease. It is believed that female sex hormones, particularly estrogen, influence the immune system, making females more susceptible to the development of lupus. The development or increased activity of lupus with pregnancy or with the use of birth control pills, instances in which estrogen levels are increased, also supports this concept.

Summary

Lupus is a disease that causes the immune system to attack healthy body tissue. The symptoms of lupus are varied and may be like those of other diseases. This makes lupus difficult to diagnose.
Several options are available to treat lupus. This makes it possible for people with lupus to remain active in life, family, and work.
Successful treatment of lupus depends on ·

Good communication with the doctor
Learning about lupus
Having regular checkups
Maintaining a positive attitude through stress management and relaxation

Your healthcare provider is available to answer any further questions you might have.