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Lupus: Causes, Symptoms, Testing and Treatment

DEFINITION
STATISTICS (USA)
TYPES OF LUPUS
CAUSES
PREGNANCY AND LUPUS
SYMPTOMS OF LUPUS
DIAGNOSIS
LABORATORY TESTS USED IN THE DIAGNOSIS OF LUPUS
WHAT TRIGGERS LUPUS?
TREATMENT/ADVERSE EFFECTS OF MEDICATION
NUTRITION AND DIET
PROGNOSIS

DEFINITION
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 auto- immune 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 "self." These antibodies, called "auto-antibodies," react with the "self" antigens to form immune complexes that build up in the tissues and can cause pain, inflammation, and further, injury to tissues. For many patients, lupus is a mild disease affecting only a few organs. For others, it may cause serious and even life-threatening problems.

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STATISTICS (USA)
According to the recent statistics (Bruskin/Goldring Research, 1994.), More people have lupus than AIDS, cerebral palsy, multiple sclerosis, sickle-cell anemia and cystic fibrosis combined. LFA (Lupus Foundation of America) research data show that between 1,400,000 and 2,000,000 people reported to have been diagnosed with lupus.

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TYPES OF LUPUS
There are three types of lupus:
(1) discoid, (2) systemic, and (3) drug-induced.

Discoid lupus is always limited to the skin identified by a rash that may appear on the face, neck and scalp. Discoid lupus is diagnosed by examining a biopsy of the rash. In discoid lupus the biopsy will show abnormalities that are not found in skin without the rash. Discoid lupus does not generally involve the body's internal organs. Therefore, the ANA test, a blood test used to detect systemic lupus, may be negative in patients with discoid lupus. However, in a large number of patients with discoid lupus, the ANA test is positive, but at a low level. In approximately 10 percent of the patient with discoid lupus can evolve into the systemic (whole body) form of the disease, which can affect almost any organ or system of the body. This cannot be predicted or prevented. Currently, treatment of discoid lupus will not prevent its progression to the systemic form.

Most often when people mention "lupus," they are referring to the systemic form of the disease. Systemic lupus(known as SLE: Systemic Lupus Erythematosus) is usually more severe than discoid lupus since it can affect almost any organ or system of the body. For some people, only the skin and joints will be involved. In others, the joints, lungs, kidneys, blood or other organs and/or tissues may be affected. Generally, the representation of the disease varies among individuals. Systemic lupus may include periods in which few, if any, symptoms are evident (remission) and other times when the disease becomes more active (flare). Individuals who progress to the systemic form probably had systemic lupus at the outset, with the discoid rash as their main symptom.

Drug-induced lupus occurs after the use of certain prescribed drugs. The symptoms of drug-induced lupus are similar to those of systemic lupus. The drugs most commonly connected with drug-induced lupus are hydralazine (used to treat high blood pressure or hypertension) and procainamide (used to treat irregular heart rhythms). However, not everyone who takes these drugs will develop drug-induced lupus. Only about 4 percent of the people who take these drugs will develop the antibodies suggestive of lupus. Of those 4 percent, only an extremely small number will develop overt drug-induced lupus. The symptoms usually fade when the medications are discontinued.

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CAUSES
The cause/causes of lupus is unknown, but environmental and genetic factors maybe involved. Scientists believe that
(1) genetic,
(2) hormone-related,
(3) unknown environmental factors (such as infections, antibiotics especially those in the sulfa and penicillin groups, ultraviolet light, extreme stress, and certain drugs) play a critical role in triggering lupus.

There is no known gene or genes which are thought to cause the illness. The research in this areas is being performed sharing certain 'gene pool'. According to the American Lupus Foundation, only 10 percent of lupus patients will have a close relative (parent or sibling) who already has or may develop lupus. Statistics show that only about 5% of the children born to individuals with lupus will develop the illness.

People of African, American Indian, and Asian origin are thought to develop the disease more frequently than Caucasian women, but the studies that led to this result are small and need further investigation.Lupus is often called a "woman's disease". However, Lupus can occur at any age, and in either sex, although it occurs 10-15 times more frequently among adult females than among adult males. The symptoms of the disease are the same in men and women. Hormonal factors may explain why lupus occurs more frequently in females than in males. The increase of disease symptoms before menstrual periods and/or during pregnancy support the belief that hormones, particularly estrogen, may be involved. However, the exact hormonal reason for the greater prevalence of lupus in women, and the cyclic increase in symptoms, is still unknown.

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PREGNANCY AND LUPUS
A question of concern to many families is whether or not a young woman with lupus should risk becoming pregnant. The current general view is that there is no absolute reason why a woman with lupus should not get pregnant, unless she has moderate to severe organ involvement (i.e., central nervous system, kidney, or heart and lungs) which would place the mother and baby at risk during pregnancy. However, there is some increased risk of disease activity during or immediately (3 to 4 weeks) after pregnancy. If a person is monitored carefully, the danger can be minimized. A pregnant woman with lupus should be closely followed by both her obstetrician and her lupus specialist.

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SYMPTOMS OF LUPUS
Although lupus can affect any part of the body, most people experience symptoms in only a few organs. That's what it makes hard to pinpoint the root of the problem. Patient often can not realize the LUPUS as the root of the cause, and tend to seek the help from non-LUPUS specialized doctors. Table 1 lists the most common symptoms of people with lupus adapted from Lupus Foundation of America. The symptoms and statistics may vary.

TABLE 1. SYMPTOMS OF LUPUS
Symptom Percentage ______________________________________________________________ Achy joints (arthralgia) 95% Fever over 100 degrees F (38 degrees C) 90% Arthritis (swollen joints) 90% Prolonged or extreme fatigue 81% Skin Rashes 74% Anemia 71% Kidney Involvement 50% Pain in the chest on deep breathing (pleurism) 45% Butterfly-shaped rash across the cheeks and nose 42% Sun or light sensitivity (photosensitivity) 30% Hair loss 27% Raynaud's phenomenon (fingers turning white and/or blue in the cold) 17% Seizures 15% Mouth or nose ulcers 12%

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DIAGNOSIS
Because many lupus symptoms mimic other illnesses, are sometimes vague and may come and go, lupus can be difficult to diagnose. Currently, there is no single laboratory test that can determine whether a person has lupus or not. Diagnosis is made by a careful review of a person's entire medical history together with an analysis of both routine laboratory tests and some specialized tests related to immune status. According to the American Rheumatism Association, a list of 11 symptoms or signs are classified to help distinguish lupus from other diseases (see Table). A person should have four or more of these symptoms to suspect lupus. It is important to notice that symptoms do not all have to occur at the same time.

TABLE. THE ELEVEN CRITERIA USED FOR THE DIAGNOSIS OF LUPUS
Criterion        			Definition
_______________________________________________________________________
Malar Rash          	Rash over the cheeks
Discoid Rash        	Red raised patches
Photosensitivity    	Reaction to sunlight, resulting in the development
                        of or increase in skin rash
Oral Ulcers         	Ulcers in the nose or mouth, usually painless
Arthritis           	Non-erosive arthritis involving two or more 
                        peripheral joints (arthritis in which the bones 
                        around the joints do not become destroyed)
Serositis           	Pleuritis or pericarditis
Renal Disorder      	Excessive protein in the urine (greater than 
                        0.5 gm/day or 3+ on test sticks)and/or cellular 
                        casts (abnormal elements the urine, derived from 
                        red and/or white cells and/or kidney tubule cells) 
Neurologic              Seizures (convulsions) and/or psychosis in the 
                        absence of drugs or metabolic disturbances which 
                        are known to cause such effects.
Hematologic Disorder    Hemolytic anemia or leukopenia (white blood count
                        below 4,000 cells per cubic millimeter) or 
                        lymphopenia (less than 1,500 lymphocytes per cubic
                        millimeter) or thrombocytopenia (less than 100,000 
                        platelets per cubic millimeter). The leukopenia 
                        and lymphopenia must be detected on two or more 
                        occasions. The thrombocytopenia must be detected 
                        in the absence of drugs known to induce it.
Immunologic Disorder    Positive LE prep test, positive anti-DNA test, 
                        positive anti-Sm test or false positive syphilis 
                        test (VDRL).  
Antinuclear Antibody    Positive test for antinuclear antibodies (ANA) in 
                        the absence of drugs known to induce it.

Adapted from: Tan, E.M., et. al. The 1982 Revised Criteria for the Classification of SLE. Arth Rheum 25: 1271-1277.

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LABORATORY TESTS USED IN THE DIAGNOSIS OF LUPUS
The interpretation of all these positive or negative tests, and their relationship to symptoms, is frequently difficult. A test may be positive one time and negative another time, reflecting the relative activity of the disease or other variables. When questions cannot be resolved, consult an expert in lupus. The first laboratory test ever devised was the LE (lupus erythematosus) cell test. When the test is repeated many times, it is eventually positive in about 90 percent of the people with systemic lupus. Unfortunately, the LE cell test is not specific for systemic lupus. The test can also be positive in up to 20 percent of the people with rheumatoid arthritis, in some patients with other rheumatic conditions like Sjogren's syndrome or scleroderma, in patients with liver disease, and in persons taking certain drugs (such as procainamide, hydralazine, and others). The immunofluorescent antinuclear antibody (ANA, or FANA) test is more specific for lupus than the LE cell prep test. The ANA test is positive in virtually all people with systemic lupus, and is the best diagnostic test for systemic lupus currently available. If the test is negative, the patient will likely not have systemic lupus. On the other hand, a positive ANA, by itself, is not diagnostic of lupus since the test may also be positive in individuals with
(1) other connective tissue diseases;
(2) individuals without symptoms;
(3) patients being treated with certain drugs, including procainamide, hydralazine, isoniazid, and chlorpromazine;
(4) individuals with conditions other than lupus, such as scleroderma, rheumatoid arthritis,
(5) infectious mononucleosis and other chronic infectious diseases such as lepromatous leprosy, subacute bacterial endocarditis, malaria, etc., and liver disease.

ANA test reports include a titer. The titer indicates how many times an individual's blood must be diluted to get a sample free of anti-nuclear antibodies. Thus, a titer of 1:640 shows a greater concentration of anti-nuclear antibodies than a titer of 1:320 or 1:160. The titer is always highest in people with lupus. Patients with active lupus have ANA tests that are very high in titer.Laboratory tests which measure complement levels in the blood are also of some value. Complement is a blood protein that, with antibodies, destroys bacteria. It is an "amplifier" of immune function. If the total blood complement level is low, or the C3 or C4 complement values are low, and the person also has a positive ANA, some weight is added to the diagnosis of lupus. Low C3 and C4 complement levels in individuals with positive ANA test results may also be indicative of lupus kidney disease. New tests of individual antigen antibody reactions have been developed which are very helpful in the diagnosis of SLE. These include the anti-DNA antibody test, the anti-Sm antibody test, the anti-RNP antibody test, the anti-Ro antibody test, and tests which measure serum complement levels. These tests can also be further explained by your physician. Physicians will sometimes also perform skin biopsies of both the individual's rashes and his or her normal skin. These biopsies can help diagnose systemic lupus in about 75 percent of patients.

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WHAT TRIGGERS LUPUS?
Many factors, yet not confirmed, can trigger the onset of the disease. Scientists have noted common features in many lupus patients such as exposure to the sun (for the sudden rash and other symptoms), infection (perhaps a cold or a more serious infection). In still other cases, a drug taken for some illness produces the signaling symptoms. In some women, the first symptoms and signs develop during pregnancy. In others, they appear soon after delivery. Many people cannot remember or identify any specific factor. According to race, there may be some hidden factors related to the gene-pool of that specific race that are susceptible to the onset of lupus. Certainly, further investigation is necessary.

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TREATMENT/ADVERSE EFFECTS OF MEDICATION
For the vast majority of people with lupus, effective treatment can minimize symptoms, reduce inflammation, and maintain normal bodily functions. Preventive measures can reduce the risk of flares. For photosensitive patients, avoidance of (excessive) sun exposure and/or the regular application of sun screens will usually prevent rashes. Regular exercise helps prevent muscle weakness and fatigue. Immunization protects against specific infections. Support groups, counseling, talking to family members, friends, and physicians can help alleviate the effects of stress. Needless to say, negative habits such as smoking, excessive consumption of alcohol, too much or too little of prescribed medication, or postponing regular medical checkups are hazardous to people with lupus.

Treatment approaches are based on the specific needs and symptoms of each person. Because the characteristics and course of lupus may vary significantly among people, it is important to emphasize that a thorough medical evaluation and ongoing medical supervision are essential to ensure proper diagnosis and treatment. Medications are often prescribed for people with lupus, depending on which organ(s) are involved, and the severity of involvement. Effective patient-physician discussions regarding the selection of medication, its possible side effects, and any changes in doses are vital. Commonly prescribed medications include

Nonsteroidal Anti-Inflammatory Drugs (NSAIDs): These medications are prescribed for a variety of rheumatic diseases, including lupus. The compounds include acetylsalicylic acid (e.g., aspirin), ibuprofen (Motrin), naproxen (Naprosyn), indomethacin (Indocin), sulindac (Clinoril), tolmetin (Tolectin), and a large number of others. These drugs are usually recommended for muscle and joint pain, and arthritis. Aspirin may cause stomach upsets for some people. This effect can be minimized by taking them with meals, milk, or antacids. The other NSAIDs work in the same way as aspirin, but tend to be less irritating to the stomach than aspirin, and often require fewer pills per day to have the same effect as aspirin.

Acetaminophen: Acetaminophen (e.g., Tylenol) is a mild analgesic that can often be used for pain. It has the advantage of less stomach irritation than aspirin, but less effective at suppressing inflammation than aspirin.

Corticosteroids: Corticosteroids (steroids), such as prednisone or dexamethasone, are hormones that have anti-inflammatory and immunoregulatory properties that are normally produced in small quantities by the adrenal gland. Corticosteroids are crucial in maintainance and treatment of lupus, However it is important to know its possible side effects to be cautious about. Side effects occur more frequently when steroids are taken over long periods of time at high doses (for example, 60 milligrams of prednisone taken daily for periods of more than one month). Such side effects include weight gain, a round face, acne, easy bruising, "thinning" of the bones(osteoporosis), high blood pressure, cataracts, onset of diabetes, increased risk of infection and stomach ulcers. As it may sound bad enough, actually corticosterioids are good anti-inflammatory and immunosupressor saving lives of many lupus patients.

Anti-malarials: The drug used in treatment of malaria are important in treating lupus. Chloroquine (Aralen) or hydroxy- chloroquine(Plaquenil) may be very useful in some individuals with lupus. They are most often prescribed for skin and joint symptoms of lupus. It may take months before these drugs demonstrate a beneficial effect. Side effects are rare, and consist of occasional diarrhea or rashes. Some anti-malarial drugs, such as quinine and chloroquine, can affect the eyes. Therefore, it is important to see an eyedoctor (ophthalmologist) regularly. The manufacturer suggests an eye exam before starting the drug and one exam every six months thereafter. However your physician might suggest a yearly exam is sufficient.

Cytotoxic Drugs: Azathioprine (Imuran) and cyclophosphamide (Cytoxan) are in a group of agents known as cytotoxic or immunosuppressive drugs primarily used in cancer treatment. These drugs act in a similar manner to the corticosteroid drugs as immune-suppressive agents. The side effects of these drugs include anemia, low white blood cell count, and increased risk of infection.

People with lupus should learn to recognize early symptoms of disease activity. In that way they can help the physician know when a change in therapy is needed. Regular monitoring of the disease by laboratory tests can be valuable because noticeable symptoms may occur only after the disease has significantly progresses. Generally, it seems that the earlier such flares are detected, the more easily they can be controlled before any possible permanent tissue or organ damages occur. Also, early treatment may decrease the chance of and reduce the time of high dose of medication. The dose of these medication and its side effects is important and need to be further characterized among Korean patient populations.

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NUTRITION AND DIET
Although much is still not known about the nutritional factors in many kinds of disease, the necessity of a well-balanced diet is not questionable. Scientists have shown that both antibodies and other cells of the immune system may be adversely affected by nutritional deficiencies or imbalances. Thus, significant deviations from a balanced diet may have profound effects on a network as complex as the immune system. It is important to realize that the well-balanced diet may help to overcome Lupus. Especially fish oil has been used only in animals with limited success and should not become the mainstay of a person's diet.

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PROGNOSIS
The idea that lupus is generally a fatal disease is simply misconception. In fact, the prognosis of lupus is much better than ever before. Today, with early diagnosis and current methods of therapy, in America, 80-90 percent of people with lupus can look forward to a normal lifespan with proper follow-up by qualified physician, regular medication(s) as prescribed, and knowledge about unexpected side-effects of a medication or a new manifestation of their lupus. Although some people with lupus have severe recurrent attacks and are frequently hospitalized, most people with lupus rarely require hospitalization. It is important to notice that there are many lupus patients who never have to be hospitalized, especially if they are careful and follow their physician's instructions. New research brings unexpected findings each year. The progress made in treatment and diagnosis during the last decade has been greater thanthat made over the past 100 years. It is therefore a sensible idea to maintain control of a disease that tomorrow may be curable.

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