Lupus

What is lupus?

Lupus is a chronic autoimmune disease that can damage any part of the body (skin, joints, and/or organs). "Chronic" means that the signs and symptoms tend to last longer than six weeks and often for many years.

In lupus, something goes wrong with the immune system, which is the part of the body that fights off viruses, bacteria, and germs ("foreign invaders," like the flu). Normally our immune systems produce proteins called "antibodies" which protect the body from these invaders.

"Autoimmunity" means your immune system cannot tell the difference between these foreign invaders and your body’s healthy tissues ("auto" means "self"). As a result, it creates autoantibodies that attack and destroy healthy tissue. These autoantibodies cause inflammation, pain, dan damage in various parts of the body.

Additional facts about lupus that you should know:
• Lupus is not contagious, not even through sexual contact. You cannot "catch" lupus from someone or "give" lupus to someone.
• Lupus is not like or related to cancer. Cancer is a condition of malignant, abnormal tissues that grow rapidly and spread into surrounding tissues. Lupus is an autoimmune disease, as described above. However, some treatments for lupus may include immunosuppresant drugs that are also used in chemotherapy.
• Lupus is not like or related to HIV (Human Immune Deficiency Virus) or AIDS (Acquired Immune Deficiency Syndrome). In HIV or AIDS the immune system is underactive; in lupus, the immune system is overactive.
• Lupus can range from mild to life-threatening and should always be treated by a doctor. With good medical care, most people with lupus can lead a full life.
• Most people who develop lupus are in in this age range: 15 to 44 years of age
• More than 16,000 new cases of lupus are reported annually across the country.
• Our research estimates that at least 1.5 million Americans have lupus. The actual number may be higher; however, there have been no large-scale studies to show the actual number of people in the U.S. living with lupus.
• It is believed that 5 million people throughout the world have a form of lupus.
• Lupus strikes mostly women of childbearing age (15-44). However, men, children, and teenagers develop lupus, too.
• Women of color are 2 to 3 times more likely to develop lupus.
• People of all races and ethnic groups can develop lupus.

What are the common symptoms of lupus?
Because lupus can affect so many different organs, a wide range of symptoms can occur. These symptoms may come and go, and different symptoms may appear at different times during the course of the disease.

The most common symptoms of lupus, which are the same for females and males, are:
• Extreme fatigue (tiredness)
• Headaches
• Painful or swollen joints
• Fever
• Anemia (low numbers of red blood cells or hemoglobin, or low total blood volume)
• Swelling (edema) in feet, legs, hands, and/or around eyes
• Pain in chest on deep breathing (pleurisy)
• Butterfly-shaped rash across cheeks and nose
• Sun- or light-sensitivity (photosensitivity)
• Hair loss
• Abnormal blood clotting
• Fingers turning white and/or blue when cold (Raynaud’s phenomenon)
• Mouth or nose ulcers

Many of these symptoms occur in other illnesses. In fact, lupus is sometimes called "the great imitator" because its symptoms are often like the symptoms of rheumatoid arthritis, blood disorders, fibromyalgia, diabetes, thyroid problems, Lyme disease, and a number of heart, lung, muscle, and bone diseases.

You play an important role in helping your doctor manage your disease. Listen to your body, ask questions and stay involved.

What causes lupus?

Many (but not all) scientists believe that lupus develops in response to a combination of factors both inside and outside the body, including hormones, genetics, and environment.

Hormones: Hormones are the body’s messengers. They regulate many of the body’s functions. Because nine of every 10 occurrences of lupus are in females, researchers have looked at the relationship between estrogen and lupus. While men and women both produce estrogen, its production is much greater in females. Many women have more lupus symptoms before menstrual periods and/or during pregnancy when estrogen production is high. This may indicate that estrogen somehow regulates the severity of lupus. However, no causal effect has been proven between estrogen, or any other hormone, and lupus. And, studies of women with lupus taking estrogen in either birth control pills or as postmenopausal therapy have shown no increase in significant disease activity. Researchers are now focusing on differences between men and women, beyond hormone levels, which may account for why women are more prone to lupus and other autoimmune diseases.

Genetics: Researchers have now identified more than 50 genes which they associate with lupus. These genes are more commonly seen in people with lupus than in those without the disease, and while most of these genes have not been shown to directly cause lupus, they are believed to contribute to it.

The chance an identical twin of someone with lupus will also develop the disease.

In most cases, genes are not enough. This is especially evident with twins who are raised in the same environment and have the same inherited features yet only one develops lupus. Although, when one of two identical twins has lupus, there is an increased chance that the other twin will also develop the disease (30% percent chance for identical twins; 5-10% percent chance for fraternal twins).

Lupus can develop in people with no family history of it, but there are likely to be other autoimmune diseases in some family members.

Certain ethnic groups (people of African, Asian, Hispanic/Latino, Native American, Native Hawaiian, or Pacific Island descent) have a greater risk of developing lupus, which may be related to genes they have in common.

Environment: Most researchers today think that an environmental agent, such as a virus or possibly a chemical, randomly encountered by a genetically susceptible individual, acts to trigger the disease. Researchers have not identified a specific environmental agent as yet but the hypothesis remains likely. While the environmental elements that can trigger lupus and cause flares aren’t fully known, the most commonly cited are ultraviolet light (UVA and UVB); infections (including the effects of the Epstein-Barr virus), and exposure to silica dust in agricultural or industrial settings.

Other examples of potential environmental triggers include:
• Ultraviolet rays from the sun and/or fluorescent light bulbs
• Sulfa drugs, which make a person more sensitive to the sun, such as: Bactrim® and Septra® (trimethoprim-sulfamethoxazole); sulfisoxazole (Gantrisin®); tolbutamide (Orinase®); sulfasalazine (Azulfidine®); diuretics
• Sun-sensitizing tetracycline drugs such as minocycline (Minocin®)
• Penicillin or other antibiotic drugs such as: amoxicillin (Amoxil®); ampicillin (Ampicillin Sodium ADD-Vantage®); cloxacillin (Cloxapen®)
• Infection, colds, or viral illnesses
• Cold or a viral illness
• Exhaustion
• Injury
• Emotional stress, such as divorce, illness, death in the family, or other life complications
• Anything else that causes stress to the body such as surgery, physical harm, injury, pregnancy, or giving birth

Can anything be done to slow or prevent the development of lupus?

Early diagnosis, as well as avoiding triggers for the disease, such as excessive exposure to the sun, can aid in the management of lupus. A rheumatologist -- a specialist in conditions of the muscles, joints, and bones -- can treat lupus with a number of medications that can effectively slow the progress of disease. However, therapy is often tailored to lessen the individual's particular symptoms.

In lupus, something goes wrong with your immune system, the part of the body that fights off viruses, bacteria, and germs.

Normally, our immune system produces proteins called antibodies that protect the body from these foreign invaders. When you have lupus, your immune system cannot tell the difference between these foreign invaders and your body’s healthy tissues, so autoantibodies are made that damage and destroy healthy tissue (auto means self and anti means against, so autoantibody means against self). These autoantibodies cause inflammation, pain, and damage in various parts of the body.

A doctor who is considering the possibility of lupus will look for signs of inflammation which include, pain, heat, redness, swelling, and loss of function at a particular place in the body. Inflammation can occur on the inside of your body (your kidneys or heart, for example), on the outside (your skin), or both.

There are many challenges to reaching a lupus diagnosis. The disease is known as "the great imitator" because its symptoms mimic many other illnesses. The symptoms of lupus can also be unclear, come and go, or change over the course of the disease.

A physician will review the following while evaluating a lupus diagnosis:

• Penicillin or other antibiotic drugs such as: amoxicillin (Amoxil®); ampicillin (Ampicillin Sodium ADD-Vantage®); cloxacillin (Cloxapen®)
• Your current symptoms
• Your laboratory test results
• Your medical history
• The medical history of your close family members (grandparents, parents, brothers and sisters, aunts, uncles, cousins)

There is no single diagnostic test for systemic lupus. The test you will hear most about is called the antinuclear antibody (ANA) test. This is not a specific test for lupus, however. In fact, a variety of laboratory tests are used to detect physical changes or conditions in your body that can occur with lupus. Each test result adds more information to the picture your doctor is forming of your illness.

Laboratory tests alone cannot give a definite “yes” or “no” diagnosis because of the following limitations:

• No single laboratory test can determine whether a person has lupus
• Test results that suggest lupus can be due to other illnesses or can even be seen in healthy people.
• A test result may be positive one time and negative another time.
• Different laboratories may produce different test results.

If multiple diagnostic criteria are present simultaneously, your physician may reach a lupus diagnosis. If, however, as is often the case, symptoms present gradually over time, the diagnosis may not be as obvious. In these cases, further consultation with a rheumatologist may be needed.

Lupus symptoms vary from one person to another. In many cases, the best treatment approach is with a health care team that will tailor treatment to your specific condition.

Choosing the right doctor

A rheumatologist, a doctor who specializes in diseases of the joints and muscles, generally treats people with lupus. If lupus has caused damage to a particular organ, other specialists should be consulted.

For instance, a dermatologist for cutaneous lupus (skin disease), a cardiologist for heart disease, a nephrologist for kidney disease, a neurologist for brain and nervous system disease, or a gastroenterologist for gastrointestinal tract disease. A woman with lupus who is considering a pregnancy needs an obstetrician who specializes in high-risk pregnancies.

Available medications

Today, physicians treat lupus using a wide variety of medicines, ranging in strength from mild to extremely strong. Prescribed medications will usually change during a person’s lifetime with lupus. However, it can take months—sometimes years—before your health care team finds just the right combination of medicines to keep your lupus symptoms under control.

There are many categories of drugs physicians use to treat lupus. However, the U.S. Food and Drug Administration or “FDA” has approved only a few specifically for lupus, which include:

• Corticosteroids, including prednisone, prednisolone, methylprednisolone, and hydrocortisone
• Antimalarials, such as hydroxychloroquine (Plaquenil®) and chloroquine
• The monoclonal antibody belimumab (Benlysta®)
• Acthar (repository corticotropin injection), which contains a naturally ocurring hormone called ACTH (adrenocorticotropic hormone)
• Aspirin

Many other medications are used "off label" to treat the symptoms of lupus, such as non-steroidal anti-inflammatory drugs (NSAIDs), immune modulating drugs (immunosuppressives), and anticoagulants.

Treatment goals

Once you have been diagnosed with lupus, your doctor will develop a treatment plan based on your age, symptoms, general health, and lifestyle. The goals of any treatment plan are to:

• Reduce inflammation caused by lupus
• Suppress your overactive immune system
• Prevent flares, and treat them when they occur
• Control symptoms like joint pain and fatigue
• Minimize damage to organs

People with lupus often require other drugs to treat conditions commonly seen with the disease. Examples include:
• Diuretics for fluid retention
• Antihypertensive drugs for high blood pressure
• Anticonvulsants for seizure disorders
• Antibiotics for infections
• Bone-strengthening drugs for osteoporosis

This information was developed by the Lupus Foundation of America, Inc. and is herewith used with permission.

Lahita R. What is Lupus? Lupus Foundation of America, Inc. Available at: https://www.lupus.org/answers/entry/what-is-lupus. Accessed January 3, 2018.

The information in this document is for general educational purposes only. It is not intended to substitute for personalized professional advice. Although the information was obtained from sources believed to be reliable, MedLink Corporation, its representatives, and the providers of the information do not guarantee its accuracy and disclaim responsibility for adverse consequences resulting from its use. For further information, consult a physician and the organization referred to herein.