SLE, or simply lupus, is a disease with a two-phase pattern. In the first phase, or flare, disease activity results in increasingly severe symptoms that require medical attention and treatment. In the second, or chronic phase, individuals may continue to have symptoms which are severe but which do not completely go away.
Lupus may also go into complete remission where the disease remains quiet for long periods of time and individuals are free of symptoms. This does not mean that lupus has been cured. All people who have lupus, including those in remission, must be careful to avoid situations which might cause the disease to

The cause of lupus is not known. The immune system, which is nature's way of protecting us from infection and cancer, works improperly in patients with lupus. Certain white blood cells, the T lymphocytes, aid in control of the immune system. In lupus, these cells fail to fulfill their regulatory function and the immune system becomes active when it is not supposed to. What starts this has not been discovered. It appears that some foreign invader, some trigger or triggers from the outside must enter the body and set this process in motion. For years the search has gone on in vain to find a virus which could act as such a trigger. We do know that sunlight and some chemicals appear to trigger lupus in some patients, though not in all. We also know that some people have an inherited tendency to develop lupus. Something in their genetic makeup makes them especially sensitive to an environmental trigger.
Lupus is a member of the autoimmune family of diseases which include rheumatoid arthritis, multiple sclerosis, juvenile diabetes, scleroderma and others. Lupus is not a form of cancer, is not related to AIDS and is not contagious.
Systemic lupus erythematosus (SLE) s the most common and serious type of lupus. The autoimmune reaction in SLE may target any tissue in the body, including the skin, muscles, joints, blood and blood vessels, lungs, heart, kidney and/or brain.
To aid in the diagnosis of lupus, the American College of Rheumatology has listed various criteria (guidelines based on symptoms and laboratory tests) to be used in identifying the disease The list contains the problems that are most typical of lupus as compared to other similar diseases. It is not meant to includeall, or even the most common problems a patient with lupus may experience. Persons with lupus most frequently suffer from fatigue, fevers, muscle and joint aching and stiffness, swollen glands, and generalized feelings of being unwell. None of these are listed as criteria for diagnosing SLE, however, because they occur in many other diseases as well.

The first of the criteria involve the skin. The best known of the skin problems in lupus is the "butterfly" rash. This is a red rash over the cheeks and the bridge of the nose (the malar area). Several other skin-related problems which commonly occur are sun sensitivity, loss of hair, red plaques (slightly raised patches of skin that have a definite border) associated with scaling and plugging of the hair follicles ("discoid" rash), hives, dryness and ulceration of the mucous membrane (mouth, nose, and vagina), and dilated and broken blood vessels. The circulation of blood to the skin and the underlying tissues is sometimes temporarily decreased in lupus, this is known as Raynaud's phenomenon. The decrease is most commonly caused by cold temperatures but can also result from psychological stress. The tips of the fingers, the tips of the toes, and occasionally the nose and the ears are affected. These areas first turn white, then blue, and finally, as the circulation returns, red. If the circulation is reduced over a long period of time, the skin may break down and ulcerations (sores) appear.
Almost all lupus patients have joint pains at some time during their illness, and many have joint inflammation (swelling, warmth, redness, pain) or arthritis. The type of arthritis associated with lupus is similar to that seen in rheumatoid arthritis but is usually not as severe and does not cause a wearing away of the bone with resulting deformity. It most commonly affects the small joints of the hands, the wrists, the knees, and the feet. Sometimes patients with lupus, especially those on corticosteroids, have involvement of the bone due to a loss of blood supply. When this occurs in the bone near a joint, degenerative arthritis may result.

Serositis is the inflammation of the delicate tissues which cover certain internal organs and line body compartments. It is quite common in lupus, most often occurring as inflammation of the covering of the lung and the lining of its compartment in the chest (pleuritis), and as inflammation of the covering of the heart (pericarditis). Movement of the involved tissues may cause chest pain, and the doctor can often hear a characteristic "rub" when listening over the inflamed areas with a stethoscope. Abdominal pain in lupus is sometimes caused by inflammation of the covering of the intestines and the lining of the abdominal cavity (peritonitis), and can imitate pancreatitis, diverticulitis, appendicitis, etc. Fluid sometimes accumulates as a result of serositis and may have to be withdrawn by a needle. In the case of pericarditis, an operation to make an opening in the covering of the heart (a "Pericardial window") may be necessary to relieve pressure on the heart from this fluid.
One of the most feared complications of lupus is involvement of the kidney. It is present to some degree in about 50% of cases. It can vary from very mildinvolvement which causes the patient no symptoms, to severe disease leading to kidney failure and the need for dialysis or kidney transplantation. Doctors detect early kidney problems by discovering protein or blood cells in the urine. They frequently ask the patient to collect 24 hour urine samples so as to be able to make better estimates of the degree to which the kidney is functioning. At times it is necessary to obtain a biopsy of the kidney to look for the presence of antibodies and kidney cell damage.

The central nervous system (CNS) may become involved by systemic lupus erythematosus. Such involvement may cause a variety of disorders including seizures, psychosis, paralysis and personality disorders. Since very similar problems might be the result of infection, hardening of the arteries, or mental illness, etc., and since there is no one test for central nervous system lupus, these disorders may be difficult for the physician to diagnose.
One of the frequent disorders in lupus is a decrease in the number of white blood cells, especially the lymphocytes. Since some of the drugs used to treat lupus can also cause a decrease in the white blood cell count, monitoring the safe use of these drugs can, at times, be difficult. The platelet is another blood cell frequently decreased in lupus. Platelets play a key role in blood clotting and having too few platelets can lead to serious bleeding disorders. Anemia, a decrease in the number of red blood cells, is almost always present in cases of active lupus. It may result from bleeding, from a bone marrow which is depressed by illness and therefore does not produce enough red blood cells, or from the destruction of red blood cells by antibodies.

When the number of certain T lymphocytes is decreased, the B lymphocytes of the lupus patient are stimulated to manufacture many different antibodies. Among these are antibodies called "autoantibodies" which target the lupus patient's own tissues. The body, in essence, attacks itself. In addition to the widely known antinuclear antibody (ANA), there are many other antibodies, the presence of which aid in the diagnosis of lupus.
Certain drugs in common use may cause antinuclear antibodies to develop and, therefore, cause a mild form of lupus known as drug-induced lupus. Drug-induced lupus is characterized by muscle pain and arthritis, fever, and pleuritis. Kidney and central nervous system involvement typically do not occur. Drugs which have been associated with drug-induced lupus include antibiotics, certain thyroid medication, and drugs used to control blood pressure, seizures, and heart rhythm. Two of the most common offenders are hydralazine (Apresoline) and procainamide (Pronestyl). Because of these drug effects, it is important to know what medications a patient diagnosed as having lupus has been taking.

Pregnancy is the occasion for special concern in the patient with systemic lupus erythematosus. While pregnancy does not appear to affect the overall life expectancy of the lupus patient, lupus may flare during pregnancy and there isan increase in premature delivery and in stillbirth among lupus patients. Therefore, it is very important for the woman with lupus to seek medical help early in her pregnancy from an obstetrician who is experienced in dealing with high-risk pregnancies.

Lupus may be difficult at times to diagnose and to distinguish from other connective tissues diseases and the symptoms may actually overlap with some of them. Rheumatoid arthritis involves a similar, though usually more severe, arthritis with morning stiffness. Patients with rheumatoid arthritis may have a positive ANA in addition to a positive test for an antibody known as the rheumatoid factor. Scleroderma (systemic sclerosis) typically is associated with Raynaud's phenomenon, arthritis and a positive ANA but is characterized by the development of remarkably tight skin. Dermatomyositis involves a facial rash and may be associated with arthritis and lung disease, but profound muscle weakness also occurs. Overlapping symptoms of lupus, derrnatomyositis, and scleroderma is not uncommon. Some of these overlap cases (including those referred to as mixed connective tissue disease) respond well to treatment and do not develop significant kidney or neurologic disease.
Some patients with lupus require very little treatment. They may need only steroid skin creams or ointments and sunscreens. Other patients may require the use of simple pain medications and mild anti-inflammatory medicines such as aspirin and NSAIDS (nonsteroidal anti-inflammatory drugs) for muscle pain or arthritis. Drugs such as hydroxychloroquine (an anti-malarial medication) are used for patients with arthritis and skin disease. Adrenal corticosteroids (steroids) given by mouth are usually reserved for the more serious cases such as those with kidney or central nervous system disorders, certain blood cell problems, or serositis. To supplement steroids or to replace them when they don't work, immunosuppressive drugs, which were originally developed for use in cancer chemotherapy but have also been found to be helpful in treating lupus, are used.

In addition to medical treatment, there are other important considerations in the treatment of lupus. Clubs and support groups are available to offer education and psychological help. Physical and occupational therapy and other methods used by rehabilitation medicine can provide pain relief and can help to maintain functioning . Psychotherapy and counselling can be an important addition to other forms of treatment and are available from different sources including psychiatrists, psychologists, and social workers. Finally, one of the most helpful aspects of the treatment program is a good doctor-patient relationship.

Signs, Symptoms, and Tests

The specific symptoms of lupus are caused by inflammation in one or more tissues of the body, and can vary widely from person to person. The following list contains the more common or classic signs and symptoms of lupus. Please note that few people would ever develop all of these conditions. A diagnosis of lupus can be made when at least four are noted in the individual's history, one of which should be a laboratory test to confirm the presence of adnormal antibodies.

- Pain in the joints of the hands, arms shoulders, feet, legs, hips or jaw which may move from joint to joint and may be accompanied by swelling, redness and heat.

- A red rash across the upper cheeks and over the bridge of the nose.

- An unusual and excessive reactions to sunlight.

- Small, usually painless, sores found on the moist lining of the nose and month.

- Chest pain that worsens when breathing in or lying down.

- Weight gain and swelling of the feet and legs which is caused by decreses in kidney function.

- Seizures or severe mental illness.

- A decrease in the number of red or white blood cells or platelets.

- Presence of certain factors in the blood, specifically the LE cell, anti-native DNA autoantibodies, anti-Sm antoantibodies or a postive test for syphillis. ( A falsely positive syphillis test indicates an abnormality in the immune response. There is no relationship between lupus and venereal diseases.)

- Presence of antinuclear antibodies ( ANA ) in the blood.

The above is not a complete listing of lupus symptoms, however, it does provide sufficient criteria for confirming a diagnosis of lupus.

Often, some generalized feelings of illness will be experienced before specific lupus symptoms appear. Individuals may describe flu-like symptoms of fatigue, aching muscles and joints, loss of appetite, fever and swollen glands. These symptoms may persist for several months and are often early warning signs of disease activity. Some of these symptoms will lessen with treatment, while others may become a feature of the chronic phase of the disease.
There are some additional symptoms that are commonly experienced. Sudden and unexplained weight loss or gain, increase in the type or severity of headaches, increase in hair loss involving the whole scalp, changes in colour of the fingers on exposure to cold hives and/or persistent high blood pressure may be signs that lupus is present and becoming active.

Other types of lupus

Discoid lupus erythematosus (DLE) and subacute cutaneous lupus (SCLE) are two types of lupus where skin rashes and sun sensitivity are the primary symptoms. DLE may cause a red scaly rash to appear on the face, scalp, ears, arms and /or chest, while in SCLE, rashes typically occur on the arms and upper body. With these types of lupus, the internal organs are spared and general health is not affected.
A small number of individuals (approximately 10%) who are diagnosed with these more limited types of lupus may later develop symptoms of systemic lupus. As well, DLE and SCLE may sometimes be present with SLE. Tests are performed to rule out SLE whenever DLE or SCLE are diagnosed.
Drug-induced lupus develops as a reaction to certain medications used to treat other medical conditions. Not everyone using these drugs develops this type of lupus but, in certain sensitive individuals, lupus symptoms will appear. Fortunately, drug-induced lupus goes away when the person stops taking the medication that triggered the lupus.

Discoid lupus is a benign, distinctive disc-shaped skin eruption. It affects only the skin and not internal organs. Discoid lupus is neither cancerous nor contagious. The initial lesion is a small or moderate-sized, coin-shaped red patch that arises spontaneously or after mild injury or exposure to sunlight. The disease spreads to other areas, but it may be confined to the exposed areas of the body, such as the face, scalp, ears, chest, and arms. The inflammation occurring on the skin may cause destruction of hair follicles and glands and thinning of the skin, with loss of normal color. These lesions may cause permanent scarring and loss of hair.

The cause of the disease is an autoimmunity. Researchers believe that changes in the skin may be caused by an autoimmune reaction of the immune system, skin injury, or an inherited factor. However, there is no clearly defined predisposition to the disease.

Who gets lupus?

Lupus can affect men, women and children of all ages. However, it develops most frequently in women between the ages of fifteen and forty-five. In this age range, lupus is eight times more common in women than in men. For individuals younger than 15 or older than 45, lupus seems to affect either sex equally.

The cause of lupus is unknown and, as yet, there is no cure. It appears there may be a hereditary factor that makes certain individuals more likely to develop the disease. There is a definite link between lupus and certain hormones, evidenced by the diseases preference for women of childbearing age, but the relationship is not completely understood. As well, some external factors such as certain medications, viruses, sun exposure, prolonged and sever stress, as well as some unknown environmental factors, are thought to trigger the onset of the disease. However, until research scientists can fully understand the functioning of the immune system, the cause of lupus will not be known.

One of the most important aspects of lupus is the individual's ability to help control her or his disease. Sun exposure, excessive fatigue, uncontrolled stress, poor diet or lifestyle habits are factors which may trigger a flare and should be avoided. A good working relationship with the physician, the willingness to take medications as prescribed, the strong support of family and friends, and membership in lupus organizations will all help to positively affect the outcome of therapy.
Lupus flares often follow the same pattern with the same combinations of symptoms reappearing. With the physician's help, the patient may be able to identify the early warning signs of a flare and then alert the physician should they appear. While false alarms are frequent, this early identification can be important as most flares are easier to treat when they first begin rather than after they have gained momentum.

The individual's education should also include knowledge of the other possible symptoms of lupus. Throughout the person's history, new symptoms may develop and those who know the warning signs will be able to bring the presence of new symptoms to their physician's attention.
Living with SLE is a challenge and an education, but people who have lupus can learn how to meet this challenge and live a full and meaningful life. It helps to know that, with close medical supervision, most people can live a relatively normal life, that remissions or quiet intervals may occur, and that lupus organizations exist to provide information and coping support.

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