What Exactly Is Lupus?

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Robert G. Lahita, M.D., Ph. D
Chief, Division of Rheumatology and Connective Tissue Diseases
St. Luke's/Roosevelt Hospital Center
Associate Professor, College of Physicians and Surgeons
Columbia University, New York, NY

1. DEFINITION
   
   Lupus is a chronic inflammatory disease that can affect 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 "self." 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 tissues, and pain.
   
   For most people, lupus is a mild disease affecting only a few organs. For others, it may cause serious and even life-threatening problems. More than 16,000 Americans develop lupus each year. It is estimated that 500,000 to 1.5 million Americans have been diagnosed with lupus.
   
   
2. TYPES OF LUPUS
   
   
   1. Discoid (Cutaneous) Lupus
      
      is always limited to the skin. It is 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 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 or "titer." In approximately 10 percent of patients, discoid lupus can evolve into the systemic form of the disease, which can affect almost any organ or system of the body. This cannot be predicted or prevented. Treatment of discoid lupus will not prevent its progression to the systemic form. Individuals who progress to the systemic form probably had systemic lupus at the outset, with the discoid rash as their main symptom.
      
      
   2. Systemic Lupus
      
      is usually more severe than discoid lupus, and can affect almost any organ or organ 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, no two people with systemic lupus will have identical symptoms. Systemic lupus may include periods in which few, if any, symptoms are evident ("remission") and other times when the disease becomes more active ("flare"). Most often when people mention "lupus," they are referring to the systemic form of the disease.
      
      
   3. 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). Drug induced lupus is more common in men who are given these drugs more often. 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.
      
      
3. CAUSE
   
   The cause(s) of lupus is unknown, but there are environmental and genetic factors involved. While scientists believe there is a genetic predisposition to the disease, it is known that environmental factors also play a critical role in triggering lupus. Some of the environmental factors that may trigger the disease are: infections, antibiotics (especially those in the sulfa and penicillin groups), ultraviolet light, extreme stress, certain drugs, and hormones.
   
   Although lupus is known to occur within families, there is no known gene or genes which are thought to cause the illness. There are recent discoveries of a gene on chromosome 1 which is associated with lupus in certain families. Previously, genes on chromosome 6 called "immune response genes" were also associated with the disease. 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.
   
   Lupus is often called a "woman's disease" despite the fact that many men are affected. 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 after puberty or after the emergence into sexual maturity. The symptoms of the disease are the same in men and women. People of African, American Indian, and Asian origin are thought to develop the disease more frequently than Caucasian women. The reasons for this ethnic selection are not clear.
   
   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 somewhat regulate the way the disease progresses. However, the exact reason for the greater prevalence of lupus in women, and the cyclic increase in symptoms, is unknown.
   
   
4. 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 her (the mother) at risk. However, there is some increased risk of disease activity during or immediately after (3 to 4 weeks) 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 doctor."
   
   As mentioned earlier in the brochure, a condition called the antiphospholipid syndrome can be secondary to lupus and may complicate pregnancy. Antibodies against specific auto antigens commonly present on coagulation factors can cause blood to clot faster than normal or in some cases, not at all. Antiphospholipid antibodies can be found in many patients with lupus and pose a particular risk to pregnant lupus patients since their presence is often associated with miscarriages.
   
   
5. SYMPTOMS OF LUPUS
   
   Although lupus can affect any part of the body, most people experience symptoms in only a few organs. Table 1 lists the most common symptoms of people with lupus.
   
   

   Symptoms			Occurrence
   Achy joints (arthralgia)			95%
   Fever more than 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 chest on deep breathing (pleurisy)			45%
   Butterfly-shaped rash across the cheeks and nose			42%
   Sun or light sensitivity (photosensitivity)			30%
   Hair loss			27%
   Abnormal Blood Clotting Problems			20%
   Raynaud's phenomenon (fingers turning white and/or blue in the cold)			17%
   Seizures			15%
   Mouth or nose ulcers			12%

   
   
   
6. DIAGNOSIS OF LUPUS
   
   Because many lupus symptoms mimic other illnesses, are sometimes vague and may come and go, lupus can be difficult to diagnose. Diagnosis is usually made by a careful review of a person's entire medical history coupled with an analysis of the results obtained in routine laboratory tests and some specialized tests related to immune status. Currently, there is no single laboratory test that can determine whether a person has lupus or not. To assist the physician in the diagnosis of lupus, the American College of Rheumatology (ACR) in 1982 issued a list of 11 symptoms or signs that help distinguish lupus from other diseases (see Table 2). This has recently been revised. A person should have four or more of these symptoms to suspect lupus. The symptoms do not all have to occur at the same time.
   
   

   Criterion	Definition
   Malar Rash	Rash over cheeks
   Discoid Rash	Red raised patches
   Photsensitivity	Reaction to sunlight, resulting in the development of or increase in skin rash
   Oral Ulcers	Ulcers in the nose or mouth, usually painless
   Arthritis	Nonerosive arthritis involving two or more peripheral joints (arthritis in which the bones around the joints do not become destroyed)
   Serositis	Pleuritis or pericarditis (inflammation of the lining of the lung or heart)
   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 Disorder	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.
   Antinuclear Antibody	Positive test for antinuclear antibodies (ANA) in the absence of drugs known to induce it.
   Immunologic Disorder	Positive anti-double stranded anti-DNA test, positive anti-Sm test, positive antiphospholipid antibody such as anticardiolipin, or false positive syphilis test (VDRL).

   
   
   
7. LABORATORY TESTS USED IN THE DIAGNOSIS OF 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 (despite the official-sounding name) and is rarely used today. 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. If the ANA test comes back negative it is considered a normal result, and it is very good evidence against lupus as an explanation for the symptoms. If the ANA test result comes back above the normal range the test is said to be positive. A positive ANA test by itself is not proof of lupus. The ANA is only a test and like a high cholesterol value, a positive ANA doesn't necessarily equate having a disease. The ANA test may also be positive in individuals:
   
   
   1. with other connective tissue diseases;
   2. without symptoms;
   3. being treated with certain drugs, including procainamide, hydralazine, isoniazid, and chlorpromazine;
   4. with conditions other than lupus, such as scleroderma, rheumatoid arthritis, infectious mononucleosis and other chronic infectious diseases such as lepromatous leprosy, subacute bacterial endocarditis, malaria, etc., and liver disease.
   
   
   Because it can be positive in conditions other than lupus, the results of the ANA test have to be interpreted in light of the patient's medical history, as well as the current clinical signs and symptoms.
   
   ANA test reports include a titer (or strength) of the antibody. 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. A high titer of this antibody (greater than 1:1280) is usually associated with rheumatic disease but has nothing to do with how active a person's lupus is. Therefore, it is not necessary to repeat the ANA in someone who has lupus. Other blood tests (complement factors C3 and C4, anti-DNA, the complete blood count, and urinalysis) are the best laboratory index of lupus activity.
   
   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.
   
   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 be further explained by your physician.
   
   Detection of antibodies to phospholipid, such as the anticardiolipin assay or a positive lupus anticoagulant can be cause for concern especially if the patient has evidence of blood clots (thromboses). The most common manifestation of this is phlebitis or inflammation of the vessels in the calves of the legs. Presence of these antibodies in the absence of any abnormal clotting may require simple aspirin therapy to mildly thin the blood. However, evidence of abnormal blood clotting may require that the patient take a blood thinner like heparin and later warfarin to prevent blockage of small and large blood vessels. When blockage occurs in the lung or the brain it can be very serious.
   
   Laboratory tests are most useful when one remembers the following information.
   
   
   • If an individual has signs and symptoms supporting the diagnosis of lupus (e.g., at least four of the American College of Rheumatology criteria), including a positive ANA, the diagnosis is confirmed and no further testing is necessary.
     
     
   • If a person has only two or three of the ACR criteria, including a positive ANA, then the ANA supports but does not confirm the diagnosis. In these cases, unless more specific tests are positive (e.g., anti-DNA, anti-Sm, anti-Ro) the diagnosis of lupus is uncertain until more clinical findings develop or other more specific blood tests, as cited above, become positive.
   
   
   Many patients may present with vague symptoms and only a positive antiphospholipid (APL) antibody or a lupus anticoagulant. A person may only have positive antiphospholipid antibodies and be diagnosed with primary antiphospholipid syndrome instead of lupus. People with primary APL syndrome might still have problems with premature clotting of blood and require treatment.
   
   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.
   
   A kidney biopsy is sometimes required if urine or blood evaluations show evidence of kidney disease. Kidney abnormalities vary with each patient. A biopsy and special preparation of the biopsy sample is required to give the doctor an idea of the degree and type of kidney injury. Using the biopsy results the doctor can tailor therapy for each individual.
   
   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.
   
   When someone has many symptoms and signs of lupus and has positive tests for lupus, physicians have little problem making a correct diagnosis and initiating treatment. However, a more common problem occurs when an individual has vague, seemingly unrelated symptoms of achy joints, fever, fatigue, or pains. Some doctors may think the person is neurotic. Others may try different drugs in the hope of suppressing the symptoms. Fortunately, with growing awareness of lupus, an increasing number of physicians will consider the possibility of lupus in the diagnosis.
   
   A patient can help the doctor by being open and honest. A healthy dialogue between the patient and doctor results in better medical care, not only for people with lupus, but for anyone seeking medical treatment.
   
   To whom should a person go for a diagnosis of lupus? Most individuals usually seek the help of their family doctor first, and this is often sufficient. However, when unresolved questions arise or complications develop, another opinion from a specialist may be advisable. The choice of specialist depends on the problem. For example, you would see a nephrologist for a kidney problem or a dermatologist for a skin problem. Most often, a rheumatologist or clinical immunologist specializing in lupus is recommended. Referrals can be made through your family doctor, the local medical society, or the local Lupus Foundation of America chapter.
   
   
8. FLARES (WHAT TRIGGERS LUPUS?)
   
   What triggers an attack of lupus in a susceptible person? Scientists have noted common features in many lupus patients. In some, exposure to the sun causes sudden development of a rash and then possibly other symptoms. In others an infection, perhaps a cold or a more serious infection, does not get better, and then complications arise. These complications may be the first signs of lupus. 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. Obviously, many seemingly unrelated factors can trigger the onset of the disease.
   
   
9. TREATMENT OF LUPUS
   
   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 are hazardous to people with lupus. These include smoking, excessive consumption of alcohol, too much or too little of prescribed medication, or postponing regular medical checkups.
   
   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:
   
   Non-steroidal Anti-inflammatory Drugs (NSAIDs): These medications are prescribed for a variety of rheumatic diseases, including lupus. Examples of such compounds include acetylsalicylic acid (e.g., aspirin), ibuprofen (Motrin), naproxen (Naprosyn), indomethacin (Indocin), nabumetone (Relafen), tolmetin (Tolectin), and a large number of others. These drugs are usually recommended for muscle and joint pain, and arthritis. Aspirin and NSAIDs may cause stomach upsets for some people. This effect can be minimized by taking them with meals, milk, antacids, or prostaglandins such as misoprostil (Cytotec). Newer NSAIDs contain a prostaglandin in the same capsule (Arthrotec). The other NSAIDs work in the same way as aspirin, but may be more potent, and patients often require fewer pills per day to have the same effect as aspirin. Many NSAIDs are now available in "over the counter" forms. Patients should be cautious about taking too much aspirin or NSAID since too many of these can reduce the blood flow to the kidney and cause problems.
   
   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 it is not nearly as effective at suppressing inflammation as aspirin.
   
   Corticosteroids: Corticosteroids (steroids) are hormones that have anti-inflammatory and immunoregulatory properties. They are normally produced in small quantities by the adrenal gland. This hormone controls a variety of metabolic functions in the body. Synthetically produced corticosteroids are used to reduce inflammation and suppress activity of the immune system. The most commonly prescribed drug of this type is Prednisone.
   
   Because steroids have a variety of side effects, the dose has to be regulated to maximize the beneficial anti-immune/anti-inflammatory effects and minimize the negative side effects. 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, stomach ulcers, hyperactivity, and an increase of appetite.
   
   Antimalarials: Chloroquine (Aralen) or hydroxychloroquine (Plaquenil), commonly used in the treatment of malaria, may also 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 antimalarial drugs, such as quinine and chloroquine, can affect the eyes. Therefore, it is important to see an eye doctor (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.
   
   Immunomodulating Drugs: Azathioprine (Imuran) and cyclophosphamide (Cytoxan) are in a group of agents known as cytotoxic or immunosuppressive drugs. These drugs act in a similar manner to the corticosteroid drugs in that they suppress inflammation and tend to suppress the immune system. The side effects of these drugs include anemia, low white blood cell count, and increased risk of infection. Their use may also predispose an individual to developing cancer later in life.
   
   Other agents like methotrexate and cyclosporin are used to control the symptoms of lupus. Both are immunomodulating drugs which have their own side effects. These drugs are still in the investigational phase for lupus. Some of these agents are used in conjunction with apheresis, a blood filtering treatment. Apheresis has been tried by itself in an effect to remove specific antibodies from the blood but the results have not been promising.
   
   Newer agents are directed toward specific cells of the immune system. These include agents which block the production of specific antibodies like those against DNA, or agents which act to suppress the manufacture of antibodies through other mechanisms. Examples of this are intravenous immunoglobulin injections which are given on a regular basis to increase platelets (particles important to coagulation).
   
   Anticoagulants: These drugs are employed to thin the blood, or in actuality to prevent blood from clotting rapidly. They range from aspirin at very low dose which prevents platelets from sticking, to heparin/coumadin which actually prevent the blood from clotting. The latter requires careful monitoring to insure that the patient is in the "therapeutic range" or that the blood is not excessively "thin". Generally, such therapy is life-long in people with lupus and follows an actual episode of clotting (embolus or thromboses).
   
   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 flared. Changes in blood test results may indicate the disease is becoming active even before the patient develops symptoms of a flare. Generally, it seems that the earlier such flares are detected, the more easily they can be controlled. Also, early treatment may decrease the chance of permanent tissue or organ damage and reduce the time one must remain on high doses of drugs.
   
   
10. NUTRITION AND DIET
    
    Although much is still not known about the nutritional factors in many kinds of disease, no one questions the necessity of a well-balanced diet. Fad diets, advocating an excess or an exclusion of certain types of foods, are much more likely to be detrimental than beneficial in any disease, including lupus. 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.
    
    There have been suggestions about various foods and the treatment of lupus. One example is fish oil. However, these diets have been used only in animals with limited success and should not become the mainstay of a person's diet.
    
    
11. PROGNOSIS
    
    The idea that lupus is generally a fatal disease is one of the gravest misconceptions about this illness. In fact, the prognosis of lupus is much better today than ever before.
    
    It is true that medical science has not yet developed a method for curing lupus and some people do die from the disease. However, people with non-organ threatening disease can look forward to a normal lifespan if they
    
    
    • follow the instructions of their physician,
    • take their medication(s) as prescribed, and
    • know when to seek help for 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. 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 than that made over the past 100 years. It is therefore a sensible idea to maintain control of a disease that tomorrow may be curable.
    
    
12. THE LUPUS FOUNDATION OF AMERICA
    
    is the nation's leading non-profit voluntary health organization dedicated to finding the causes and cure for lupus. Our mission is to improve the diagnosis and treatment of lupus, support individuals and families affected by the disease, increase awareness of lupus among health professionals and the public, and find the causes and cure. Research, education, and patient services are at the heart of LFA's programs.
    
    The Lupus Foundation of America is the only nationwide organization exclusively serving individuals, families and friends affected by lupus. The LFA has local chapters and support groups throughout the United States, as well as international affiliates around the world. Contact the LFA or the chapter that serves your area to find out how you can become involved in our mission.
    
    

LINKS FOR MORE INFORMATION ON LUPUS

The Hamline University has a Lupus Home Page which is very informative:

Lupus Home Page

Cyndee Miller has been living with Lupus for 30 years.
See what she has to say about Lupus:

Cyndee Miller's Home Page Dedicated to Lupus Awareness

Embrace the Wolf

Rest Ministries: Ways to Find Support (Support Newsletter, Pen Pals, Email Support Group, Parenting Support, Daily Devotional, Bulletin Board, New Chat Room, New Resources)

Rest Ministries: Ways to Find Support

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