Definition: Defining the Meaning of Lupus
Systemic lupus erythematosus, often abbreviated as SLE or lupus, is a chronic inflammatory disease that occurs when your body's immune system attacks your own tissues and organs. There are many kinds of lupus. The most common type is systemic lupus erythematosus (SLE), which affects many internal organs in the body. SLE most often harms the heart, joints, skin, lungs, blood vessels, liver, kidneys, and nervous system. Childhood systemic lupus erythematosus generally presents between the ages of 3 and 15, with girls outnumbering boys 4:1, and typical skin manifestations being butterfly eruption on the face and photosensitivity.
An Introduction to Lupus
Lupus is an autoimmune disease. Lupus can affect different parts of the body; to include the heart, lungs, brain, kidneys, skin, and joints such as elbows, knees, or hips. In a person unaffected by Lupus, the immune system produces proteins called, 'antibodies,' in order to protect the body from bacteria, antigens, and viruses.
For a person who has Lupus the immune system cannot differentiate between antigens and the tissues and cells in the body. Their immune system creates antibodies that fight against it called, 'auto-antibodies,' and they cause pain, inflammation, and damage different parts of the body.
The main feature of Lupus is inflammation, and it is characterized by redness, heat, swelling, loss of function and pain outside or inside the body or both. Many people experience Lupus as a mild disease that affects a few organs only; yet others who have Lupus have serious or life-threatening issues. Data regarding Lupus is somewhat limited, but studies on it suggest that there are more than sixteen thousand Americans who develop Lupus every year.
An estimate by the Lupus Foundation of America (LFA) suggests that there are between 1.5 and 2 million Americans who are living with a form of Lupus; the number may be higher in actuality. Ninety-percent or more of the people living with Lupus are women, and of these women the ones experiencing the symptoms and being diagnosed with it are of child-bearing age; between 15 and 45 years old. In America, African Americans, Asians, Latinos, and Native Americans are more likely to have Lupus than Caucasians.
Symptoms of Lupus
Although most people with Lupus experience symptoms in a few organs only, Lupus can affect any part of the body. Ninety-five percent of people with Lupus experience achy joints, or, 'Arthralgia.' Ninety-percent experience fevers of more than one-hundred degrees, and arthritis or swollen joints. Eighty-one percent experience long-term or extreme fatigue. Skin rashes are something that seventy-four percent of people with Lupus encounter, and seventy-one percent experience anemia.
Other symptoms experiences by persons with Lupus include Kidney issues (50%), Chest pain (45%), butterfly-shaped rashes on the nose or cheeks (42%), sun/light sensitivity (30%), hair loss (27%), blood clotting issues (20%), Raynaud's Phenomenon - fingers turning white or blue in the cold (17%), seizures (15%), and mouth or nose Ulcers (12%). If you experience several of these symptoms you should contact your doctor.
Potential Causes of Lupus
Medical science does not know what causes Lupus at this time. There are; however, some different genetic and environmental factors involved. Extreme stress, ultraviolet light, some specific drugs, hormones, and antibiotics; particularly antibiotics from the penicillin and sulfa groups, are all environmental factors that may lead to Lupus.
Many members of the scientific community believe that there is a genetic predisposition on the part of some people to Lupus that occurs in families. A particular gene or genes associated with Lupus have not been identified, with the exception of a certain gene on chromosome one that is associated with Lupus and some families. There were some genes on chromosome 6 that were previously associated with Lupus; they are called, 'immune response genes.' Of all the people who have Lupus, only ten percent have a parent or sibling that may develop it, or already has it. Around five percent of the children born to people who have Lupus go on to get Lupus themselves.
Lupus does not only affect women, contrary to what many people believe; there are a number of men that are affected by Lupus. People of either sex, at any age can be affected by Lupus, but it does occur ten to fifteen times more often in adult women than men after puberty. Hormones may explain why women get Lupus more than men. There is an increase in the symptoms of Lupus prior to menstrual periods and/or during pregnancy, and this supports the idea that hormones such as estrogen and others might affect the progression of Lupus. There is still no understanding of the exact reasons behind the greater rate of Lupus in women than men.
The Lupus Diagnosis
Lupus symptoms can mimic a number of other illnesses, they are transient at times or vague, and this can make Lupus hard to diagnose. Laboratory testing, review of the person's complete medical history, along with specialized testing concerning the person's immune status, can help to reach a decision of whether or not Lupus is the correct diagnosis. In the medical field today there is no one single laboratory test that can tell if a person has Lupus or not.
The American College of Rheumatology (ACR) has issued a list of eleven symptoms or signs that can help to differentiate Lupus from other diseases. If a person has four or more of them, even if they do not occur at the same time, Lupus is something that should be investigated further with a doctor. These eleven symptoms and signs are as follows:
Symptoms and Signs of Lupus
Malar Rash: A Malar Rash is a rash that appears over the cheeks.
Discoid Rash: A Discoid Rash is a red, raised rash that appears in patches.
Photosensitivity: Photosensitivy is a reaction to sunlight that results in the development of, or an increase in, a skin rash.
Oral Ulcers: Oral Ulcers may appear in either the nose or the mouth and are usually painless.
Arthritis: The form of arthritis that is suspect in association with Lupus is Non-erosive Arthritis, and involves two or more peripheral joints. This form of arthritis is one where the bones around the joints do not become destroyed.
Serositis: Serositis, Pleuritis, or Pericarditis are symptoms of Lupus that involve inflammation of the lining of the heart or lungs.
Renal Disorder: Renal Disorder involving excess proteins in urinary output that is greater than 0.5 gm per day or 3+ on test sticks. It may also involve cellular casts, which are abnormal elements in the urine that are derived from white or red cells or both, or from kidney tubule cells.
Neurologic Disorders: Neurologic Disorders involving either psychosis, or seizure activity when metabolic disturbances or drugs that are known to cause them are absent, are a potential sign of Lupus.
Hematologic Disorders: There are several Hematologic Disorders that may be signs of Lupus. Hymolytic Anemia or Leukopenia, where the white blood cell count falls below four-thousand cells per cubic millimeter are potential signs. Lymphopenia, where less than one-thousand five hundred lymphocytes per cubic millimeter are present in the blood, is another. Thrombocytopenia, where there are less than one hundred-thousand platelets per cubic millimeter in the blood, is still another potential sign of Lupus. With Leukopenia and Lymphopenia, they must be detected on at least two or more occasions. Some drugs can induce Thrombocytopenia, so to be considered a potential sign of Lupus it must be detected in the absence of any drugs that might induce it.
Antinuclear Antibody: Antinuclear Antibodies can also be induced by certain drugs, and determining if any of these drugs are present first is important. A positive test for Antinuclear Antibodies may be a sign of Lupus.
Immunologic Disorder: Testing for an Immunologic Disorder involves several things; a positive anti-Sm test, a positive Antiphospholipid Antibody - like Anticardiolipin, a positive anti-double stranded anti-DNA test, or a false positive syphilis test.
Lupus and Treatment
Treatment can effectively minimize the symptoms and inflammation associated with Lupus for most people, as well as helping to maintain their bodily functions. There are some preventative measures that people with Lupus can take to reduce the risk of the symptoms of Lupus. People with Lupus who are photosensitive can avoid exposure to the sun or use sun screen in an effort to avoid rashes. Muscle fatigue and weakness can be prevented to a degree through regular exercise. In order to avoid some specific infections, persons with Lupus can get immunized against them.
Stress can become an issue for many people with Lupus, and to deal with stress they can reach for support groups, family members, friends, counseling, and physicians for support. In an effort to maintain as high a level of health as possible, it is wise for persons with Lupus to discontinue negative habits such as smoking, excessive drinking of alcoholic beverages, postponing medical checkups, or taking too little or too much of prescription medications.
Every person with Lupus is an individual, and treatment approaches for Lupus are equally individualized. The characteristics of Lupus vary significantly between individuals with Lupus, so it is important to receive a complete, thorough, and ongoing medical supervision of the condition in order to ensure both proper diagnosis and treatment.
Depending on which organs are involved, different medications may be prescribed according to the severity of the symptoms. An effective patient-physician relationship and discussions about the kinds of medications used and potential side-effects, as well as the need for any changes in the dosage of medications, is essential. There are several Non-Steroidal Anti-inflammatory Drugs (NSAID's) that are commonly used to aid with symptoms of Lupus.
Non-steroidal Anti-inflammatory Drugs (NSAID's) and Lupus
Some of the more common NSAID's used to help with Lupus symptoms include Aspirin, Ibuprofen, Indomethacin, Naproxen, Tolmetin, Nabumetone, and several others. These medications are also used for arthritis, muscle, and joint pain. One of the issues with these medications is that they may cause stomach upset, although this side-effect can sometimes be prevented through taking them with milk, antacids, meals, or prostaglandins like misoprostil, also known as, 'Cytotec.' Some of the newer NSAID's have a prostaglandin in the same capsule as the NSAID; one brand is, 'Arthrotec.'
Many NSAID's perform in much the same way as Aspirin but are stronger, requiring people to take fewer capsules or pills each day in order to receive the same benefits as they would from more Aspirin. A number of NSAID's are available over the counter in stores, and people with Lupus should be careful not to take more Aspirin or NSAID's than recommended because overuse of them can slow down blood flow to the kidneys, causing problems.
Additional Treatments for Lupus
Acetaminophen: Acetaminophen is an analgesic used for pain that can cause less stomach pain than Aspirin or NSAID's. It does not have the inflammatory suppressing abilities of Aspirin, unfortunately.
Corticosteroids: Corticosteroids are hormones that have both immuno-regulatory and anti-inflammatory properties; they are usually created in minor quantities in the human adrenal gland and control various metabolic functions. Synthetic Corticosteroids help to reduce immune system activity and inflammation; the most commonly prescribed one is Prednisone. Steroids can have various side-effects and the dose has to be regulated in order to achieve the desired anti-inflammatory and anti-immune effects desired while keeping any undesirable side effects at a minimum. People who take steroids at high doses over a long period of time are most likely to experience negative side-effects. Some of the undesired side-effects include acne, a round face, weight gain, stomach ulcers, bruising easily, high blood pressure, osteoporosis, diabetes, cataracts, hyperactivity, increased appetite and an increased risk of infection.
Antimalarials: Hydroxychloroquine or Chloroquine, usually used to treat malaria, can be used to treat some people who have Lupus as well; mostly people with Lupus who have joint and skin symptoms. Unfortunately, it can take months before the benefits of taking these medications are proven. On rare occasion a person taking these medications may experience rashes or diarrhea. Other Anti-malarial medications like Quinine may affect a person's eyes, so if they are used it is important to see an ophthalmologist on a regular basis. The manufacturer of these medications has suggested that people who are about to start taking these medications should visit an ophthalmologist prior to taking them, and then have an eye exam every six months afterward.
Immuno-modulating Drugs: Immuno-modulating Drugs include Azathioprine and Cyclophosphamide, and are part of a group of medications referred to as, 'Cytotoxic,' or, 'Immunosuppressive,' medications. They are somewhat like Corticosteroid medications because they reduce inflammation and the immune system; they also have side-effects. Some of the side-effects of these medications include an increased risk of infection, anemia, and the potential for a low white blood cell count. Using these medications may also predispose a person to cancer at a later point in life.
Methotrexate and Cyclosporine: Methotrexate and Cyclosporine are immuno-modulating medications that are under an investigational phase in the treatment of Lupus and are used along with Aphaeresis, which is a blood-filtering treatment. Used by itself, Aphaeresis has shown results that have not been promising. Like other medications, Methotrexate and Cyclosporine also have side-effects. There are other things referred to as, 'agents,' that are being used that are directed towards specific cells in the immune system in the fight against Lupus. Some of these agents block the production of certain antibodies; others suppress the creation of antibodies through additional means. An example of this includes immunoglobulin shots that are given regularly in order to increase a person's platelet count; something that is important to the process of coagulation.
Anticoagulants: Anticoagulants are used to help thin blood and prevent it from clotting too fast. Aspirin, taken at a low dose, prevents platelets from clotting, as does Heparin or Coumadin. Coumadin is a medication that needs to be watched closely to make sure that the person taking it stays within a therapeutic range, and to make sure that their blood is not too thin. Usually therapy of this kind is a life-long form for people with Lupus. This form of therapy commonly is started after an incident where the blood has clotted; called, 'Thromboses,' or, 'Embolus.'
The ability to recognize early symptoms of disease activity is something that persons with Lupus should learn to do because they can assist their doctor when a change in their therapy is needed. Laboratory testing in the process of regularly monitoring Lupus is very valuable because symptoms might only happen after a noticeable flare, and lab tests can show if the disease is changing to a more active state prior to the person developing symptoms. The sooner a flare is detected, the sooner it can be controlled, and early treatment can decrease risks of permanent organ or tissue damage. Early treatment can also reduce the amount of time a person needs to stay on larger dose of medications.
Lupus is Latin for wolf.
In the 18th century, when lupus was just starting to be recognized as a disease, it was thought that it was caused by a wolf's bite. This may have been because of the distinctive rash characteristic of lupus. (Once full-blown, the round, disk-shaped rashes heal from the inside out, leaving a bite-like imprint.)
The first mechanism may arise genetically.
Research indicates SLE may have a genetic link. SLE does run in families, but no single causal gene has been identified. Instead, multiple genes appear to influence a person's chance of developing lupus when triggered by environmental factors.
Statistics: U.S. Lupus
- About 5 percent of the children born to individuals with lupus will develop the illness.
- 20 percent of people with lupus will have a parent or sibling who already has lupus or may develop lupus.
- The Lupus Foundation of America estimates that 1.5 million Americans, and at least five million people worldwide, have a form of lupus.
- Although lupus can strike men and women of all ages, 90 percent of individuals diagnosed with the disease are women. Most people will develop lupus between the ages of 15-44.
- Lupus is two to three times more prevalent among women of color - African Americans, Hispanics/Latinos, Asians, Native Americans, Alaska Natives, Native Hawaiians and other Pacific Islanders - than among Caucasian women.
- In approximately 10 percent of all cases, individuals will have symptoms and signs of more than one connective tissue disease, including lupus. A physician may use the term "overlap syndrome" or "mixed connective tissue disease" to describe the illness.
- From 1979 to 1998, the annual number of deaths with lupus as the underlying cause increased from 879 to 1,406. Crude death rates increased with age (35% were in 15-44 year age group), among women (5x higher than in men), and among blacks (3x higher than in whites). Death rates were highest and increased the most over time among black women aged 45-64 years.
- It is difficult to determine the annual number of new lupus cases, or the number of individuals who die from health complications of the disease. However, due to improved diagnosis and disease management, most people with the disease will go on to live a normal life span. However, it is believed that between 10-15 percent of people with lupus will die prematurely due to complications of lupus.
- The U.S. Centers for Disease Control and Prevention issued a report in May 2002 which indicated that deaths attributed to lupus increased over a 20-year period, particularly among African American women ages 45-64. However, it is not clear if the rise is the result of an actual increase in lupus mortality or better identification and reporting of deaths due to complications of the disease. Trends in Deaths from SLE - United States, 1979 - 1998
- Systemic lupus accounts for approximately 70 percent of all cases of lupus. In approximately half of these cases, a major organ, such as the heart, lungs, kidneys or brain, will be affected. Cutaneous lupus (affecting only the skin) accounts for approximately 10 percent of all lupus cases. Drug-induced lupus accounts for about 10 percent of all lupus cases and is caused by high doses of certain medications. The symptoms of drug-induced lupus are similar to systemic lupus; however, symptoms usually subside when the medications are discontinued.