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Lupus
Disease Series
Arthritis Foundation of America

What is Lupus?

Systemic lupus erythematosus (sis-TEM-ick LOO-pus er-i-THE-mah-TOE-sis), also called lupus (or SLE for short), is a rheumatic disease. Rheumatic diseases affect joints, muscles and other parts of the body.

Lupus is both inflammatory and chronic. Inflammation refers to a body reaction resulting in pain, heat, redness and swelling. Chronic means long-lasting, possibly for the rest of one’s life. However, chronic does not necessarily mean that you will constantly experience symptoms. Nearly all people with lupus have changes in disease activity known as flares and remissions. A flare is a period of worsening inflammation. A remission is a period during which no signs or symptoms of lupus are present. On rare occasions a person may have a complete of long-lasting remission.

Several forms of lupus are listed in the following sections.

Systemic Lupus

Approximately 70 percent of the people who have lupus have systemic lupus. A systemic disease is one in which several different parts of the body may be affected. In systemic lupus, these include the skin, kidneys, nervous system, lungs, heart and/or blood-forming organs. Of those people with systemic lupus, about half have the form that can damage the body’s internal organs and half have the non-organ-threatening form.

Discoid Lupus

Approximately 15 percent of people with lupus have cutaneous (cue-TANEW-ee-us) lupus, also called discoid (DIS-coyd) lupus. This is a disease of the skin that’s often chronic and can sometimes lead to scarring. It may be limited to the skin and mot be associated with disease in any other body system.

Drug-Induced Lupus

Approximately 10 percent of people with lupus have drug-induced lupus. This means that lupus occurs because of a reaction to one or more drugs. Symptoms often disappear when the person stops taking the drugs that caused the reaction.

Crossover, or Overlap, Syndromes

About five percent of people with systemic lupus have crossover syndromes. This means that they have features that overlap with another rheumatic disease, such as rheumatoid arthritis or myositis. Because these people have a combination of symptoms, their disease may not fit into traditional diagnosis categories.

What Causes Lupus?

The cause of lupus is unknown. Doctors and scientists agree that it is an autoimmune (aw-toe-im-MUNE) disease. The immune system is your body’s natural defense against antigens, which are substances that your body regards as foreign. Bacteria and viruses are examples of antigens. The immune system fights off antigens by creating antibodies, which are special types of blood protein whose job it is to attack antigens and render them harmless.

Occasionally, the immune system does not function properly and loses the ability to distinguish between its own body cells and antigens. Instead of fighting antigens, the antibodies mistakenly fight the body’s own cells. This is referred to as an autoimmune response (auto means self, and this is a response directed against the body’s own cells).

In an autoimmune disease, antibodies that are directed against the body’s own cells are called autoantibodies. Antinuclear antibodies are autoantibodies that react against the nucleus (core) of the body’s own cells when these cell parts are mistaken for foreign substances.

The damage that occurs may take the form of inflammation and injury to body tissues and organs. Such damage may result in lupus symptoms.

Sometimes medications taken for other conditions, like high blood pressure, certain heart problems, seizures and psychiatric problems such as severe depression, may cause many of the symptoms and abnormal blood test results associated with lupus. Symptoms of such drug-induced lupus almost always go away when these medication are stopped. However, it may take several weeks to months for the symptoms and abnormal blood test results to disappear.

Can Lupus Be Inherited?

Lupus affects women about eight to ten times as often as men. In most cases, symptoms first appear in women of childbearing age (10-45). However, lupus can occur in young children or in older people. It occurs more often in African-Americans than in Caucasians. Some data suggests that it also might occur more frequently in Asian and Hispanic populations than in Caucasians.

Some studies suggest that certain people may inherit the tendency to get lupus. This conclusion stems from the fact that new cases of lupus may be more common in families in which one member already has the disease. However, there is no evidence that lupus is directly passed on from mother to daughter. Some scientists believe that perhaps a virus or other environmental substance may trigger the genetic tendency to develop lupus and bring on the symptoms of the disease.

What Happens in Lupus?

The is no uniform pattern of symptoms when lupus is active. You may develop several of the symptoms mentioned in this section or just a few. Each person is different, and what is true about someone else’s disease may not apply to you.

The following classification of 11 symptoms helps doctors tell the difference between people who have lupus and people who have other connective tissue disorders.

Eleven Main Symptoms

The 11 main symptoms of lupus include:

  - malar (MAY-lar) rash (a butterfly-shaped rash over the cheeks and across the bridge of the nose);

  - discoid rash (scaly, disk-shaped sores on the face, neck and/or chest);

  - photosensitivity (sensitivity to ultraviolet light);

  - oral ulcers (sores on the tongue, inside the mouth and/or in the nose);

  - arthritis (pain, stiffness and swelling in the joints);

  - serositis (SEE-row-SY-tis) (inflammation of the lining around the heart, lungs and/or abdomen, causing pain when you move     and/or breathe and often accompanied by shortness of breath);

  - kidney problems (can be mild with no early symptoms. Severe cases usually are associated with symptoms such as edema,     which is a swelling of the legs that may occur if large amounts of protein leak from the kidneys into the urine. Other severe     symptoms can include extreme fatigue, high blood pressure and loss of appetite.);

  - central nervous system problems (in a very small percentage of people, seizures or memory loss may develop, but they rarely     persist);

  - blood problems (can include anemia - ah-Nee-me-ah - a condition in which you have fewer red blood cells, causing weakness     and paleness or even shortness of breath; low white blood cell count; low platelet count; or an increased tendency to form blood     clots, which is called the antiphospholipid or anti-cardiolipin syndrome);

  - problems with the immune system (which may increase the risk of infection);

  - antinuclear antibodies (autoantibodies that react against the nucleus (core) of the body’s own cells when these cell parts are     mistaken for antigens).

Other Symptoms

People with lupus may experience additional symptoms. Fever, weakness, fatigue or weight loss may be among the first signs of illness.

Sometimes the fingers, toes, nose and/or ears become unusually sensitive to cold and will turn blue when exposed to the cold. This color change is called Raynaud’s (Ray-NODES) phenomenon.

Other common symptoms of the illness include muscle aches, swollen glands, lack of appetite, low-grade fever, hair loss and abdominal pain that may be accompanied by nausea, vomiting and diarrhea.

About 10 to 15 percent of people with lupus also have Sjogren’s (SHOW-grens) syndrome. This is a chronic disorder that causes dry eyes and a dry mouth. In women it also can include dryness in the vagina.

Sometimes people with lupus experience depression or an inability to concentrate. Rapid changes in mood or unusual behavior may occur for any of the following reasons:

  - The changes may be due to inflammation from the disease itself.

  - The changes may be a normal reaction to the changes in appearance and energy caused by the disease.

  - The changes may be due to side effects of the medications used to treat the disease. You and your doctor should discuss your     symptoms, especially when new ones develop or chronic ones worsen. Remember that lupus can take many forms. Although     people with lupus can have many symptoms, most people only have a few problems that tend to occur in a repeated pattern.     Many people with lupus do very well without having major problems.

How Is Lupus Diagnosed?

It sometimes is difficult to diagnose lupus, particularly in people with mild symptoms of the disease. This is because there is no single set of symptoms, pattern of disease of set of tests to diagnose all who have lupus.

Your doctor will begin the diagnostic process by asking you many questions and doing a physical examination. Certain tests will be performed, such as a blood count to see if you have too few red blood cells or platelets (blood cells that help to control bleeding and clotting).

If your doctor thinks you may have lupus, a blood test called an ANA usually will be done. An ANA detects a group of autoantibodies found in the blood of almost all people with lupus. These autoantibodies react with antigens in the nuclei of cells. Having these antibodies suggests that an autoimmune illness may be present, although some people test positive with little of no evidence of serious disease.

Other laboratory tests may be helpful in the diagnosis of certain related rheumatic diseases. The names of the following tests are abbreviations of some of the tests used to help confirm diagnoses:

  - anti-dsDNA;
  - anti-Sm;
  - anti-Ro/SS-A; and
  - anti-RNP.

Complement tests (C3, C4, CH50, CH100) measure the amount of complement proteins circulation on the blood. A low complement level suggests that an active inflammatory process is occurring because of the lupus. These tests usually are used to diagnose or monitor people with active lupus, especially if their kidneys are affected.

Other blood tests may be done for diagnosis and as an aid in following disease activity. Blood chemistry tests can help determine whether organs such as the kidneys and liver are functioning normally. A sedimentation rate (ESC) or C-reactive protein (CRP) also may be used to measure inflammation levels.

Because kidney problems often occur, you may have a urinalysis (you-rih-NAL-ah-sis), which is an examination of your urine. You may be asked to collect for analysis all the urine you pass in a 24-hour period. If kidney involvement is suspected, you may have a biopsy (BUY-op-see) performed, during which a small piece of tissue from one of your kidneys is removed and examined with special tests.

A chest X-ray may be taken to see whether your longs of heart are involved. An electrocardiogram and echocardiogram also may help determine if there id any heart involvement.

In a few people with lupus, all the laboratory tests may be normal, despite other evidence of the disease. In other people, they may feel fine, but the results of laboratory tests may suggest significant lupus activity.

How is Lupus Treated?

The treatment plan for lupus includes taking medications to reduce the inflammation or reduce that activity of the immune system, balancing rest with exercise and eating a proper diet.

Lupus is an unpredictable disease. Signs of the disease appear and disappear, sometimes for no apparent reason. Because lupus assumes so many different forms and can change, finding the right balance of treatment for you may take time. Once an effective treatment program has been started, continue it faithfully. If your symptoms change, let our doctor know so that you can work together to modify your program.

Medication

Medication is a necessary part of treatment for most people with lupus. Until you and your doctor can identify your flare pattern, the type and amount of medications prescribed may frequently be changed.

Changing how much medicine you take or how often you take it is a decision to be made after talking with your doctor. Many medications have a four- to six-week time lag before you experience the greatest benefits. Therefore, changing your schedule on your own or not taking a drug you doctor has prescribed could have harmful effects. It also will make it more difficult for your doctor to evaluate what is happening in your disease and how effective the medication is in reducing inflammation or reducing the activity of the immune system. If you start taking a drug without medical advice, it could harm you, either by interacting with other medications prescribed for you or by causing side effects that might even mimic some of the symptoms of your lupus. Page2