Lupus

Overview

What Is It?

Systemic lupus erythematosus (SLE) is a chronic, inflammatory disorder of the immune system that affects several body systems simultaneously.

Achy, swollen joints; fever; skin rashes; fatigue—these are some of the more typical symptoms of systemic lupus erythematosus (SLE), a chronic, inflammatory disorder of the immune system that affects several body systems simultaneously. Approximately 1.5 million people in the United States have some form of lupus, according to the Lupus Foundation of America.

SLE is classified as an autoimmune disorder because the body's immune system, which normally fights harmful bacteria and viruses, also targets healthy tissue, sometimes including skin, joints, kidneys, brain, heart and lungs. The condition can result in immediately serious or life-threatening problems or in chronic low-grade symptoms, such as fatigue and muscle aches, which affect the quality of life.

In Latin, lupus means wolf, and erythematosus means redness. Physicians who first described the disease thought that one of its characteristic rashes looked like a wolf bite. The terms "SLE" and "lupus" are often used interchangeably, but there are several kinds of lupus, including:

• Systemic lupus erythematosus (SLE) can affect almost any organ or system in the body. In some people with systemic lupus, only the skin and joints are involved; in others, the joints, lungs, kidneys, blood or other organs and/or tissues are all affected. Any two people with systemic lupus will be unlikely to have all the same symptoms. Systemic lupus may include remission periods, during which few or no symptoms are evident, and "flares," when the disease becomes active.
• Cutaneous lupus erythematosus includes discoid lupus (DLE) as well as other subsets of cutaneous lupus erythematosus. DLE involves the development of lesions on the face, scalp, ears or other areas often those exposed to the sun. Lesions can also occur in the mouth, nose or vagina. The lesions are abnormally red, hard bumps or plaques. They may include an overgrowth of scaly tissue, plugged hair follicles and abnormally widened small blood vessels. Thinning of the healing skin can be seen in active lesions as well as part of atrophic scarring. There can be a loss of color in the skin, called dyspigmentation in older lesions. If the condition involves the scalp, there may be permanent scarring and loss of hair. Many people have DLE without SLE. In approximately 10-20 percent of these cases, DLE later progresses to the more severe SLE. This is more likely to happen in patients with lesions both above and below the neck.
• Drug-induced lupus is caused by certain medications, the most common being: procainamide (Pronesyl), used for heart rhythm abnormalities, and hydralazine (Hydra-Zide), used for high blood pressure. Drug-induced lupus usually doesn't affect the kidneys or central nervous system and typically improves within six months of when the drug is discontinued. There are also specific medications, such as hydrochlorothiazide and terbenifine, that can induce a specific form of cutaneous lupus, subacute cutaneous lupus.
• Neonatal lupus, a very rare condition in a small percentage of newborns whose mothers have lupus, results from the passage of autoantibodies from the mother to her baby, most commonly anti-Ro/SSA or anti-La/SSB, which can affect the skin, heart and blood of the fetus and newborn. The most common symptom of neonatal lupus is an uncomplicated rash that appears within the first several weeks of life and may persist for about six months before disappearing. Less frequently, fetuses with neonatal lupus develop a congenital heart block. However, many of these babies do well with a pacemaker at birth. If a fetal heart condition is identified early enough during pregnancy, it might be possible to treat it with steroids or immunosuppressive drugs. Hydroxychloroquine may be used to help prevent cardiac involvement.

In general, women are far more likely than men to develop autoimmune disorders, and SLE certainly fits that paradigm, occurring up to 10 times more frequently among adult women than among men. It is also more common in African-Americans, American Indians and Asians than in Caucasians. Although lupus can develop at any age, it is usually diagnosed in women during their childbearing years. Cutaneous lupus occurs more frequently in women, but the ratio of women to men is somewhat lower than with SLE.

If you have a parent, child or sibling with lupus, your risk of developing the disease is somewhat higher, although your health care professional probably won't test you for the disease unless you develop symptoms. There is no known cure for SLE, but there are treatments designed to minimize symptoms and effects.

Lupus seems to result from a combination of genes and environment. Scientists think that people may inherit a predisposition to developing lupus but not lupus itself. People who inherit the predisposition to the disease may develop lupus after they come in contact with something that triggers it, such as a virus or medication.

The number and type of lupus symptoms vary widely among patients. Symptoms also tend to wax and wane, with patterns of inactive disease bracketed by lupus "flares."

Diagnosis

Diagnosing systemic lupus erythematosus (SLE) is not easy. Sometimes the disease announces itself decisively with a flare resembling an infection, but more often the early stages of the disease produce only vague symptoms such as aches, rashes or fatigue, which gradually worsen over time. Lupus symptoms can be easily confused with those of other diseases.

Symptoms of lupus depend on which body systems are affected. The most common symptoms include:

• arthralgia (joint pain)
• arthritis (joint warmth, swelling and redness)
• unexplained fever (over 100 degrees Fahrenheit)
• extended or severe fatigue
• skin rashes
• anemia (abnormally low red blood cell count)
• kidney damage
• alopecia (hair loss)

Less common symptoms include:

• pleurisy (chest pain when breathing deeply, caused by inflammation of the lungs' lining)
• photosensitive rashes that appear after exposure to sunlight
• baldness on areas of the scalp
• Raynaud's syndrome (fingers turning white or blue in the cold)
• seizures, psychosis and other neuropsychiatric problems
• mouth or nose ulcers
• pericarditis (chest pain caused by inflammation of the heart lining)
• phlebitis (blood clots)

Consult a health care professional as soon as possible if you have any of the symptoms listed above, whether singly or in combination. Multiple systemic symptoms indicate a stronger likelihood that lupus is the culprit, and a rheumatologist should evaluate you.

An evaluation for lupus consists of a thorough medical history, a physical examination and laboratory tests.

An initial diagnostic and disease activity screen usually includes a complete blood count, liver and kidney tests, blood tests for autoantibodies (increased antibodies that target healthy tissues and are an indicator of autoimmune disease), skin biopsy, urinalysis (to detect possible kidney disease), blood chemistry work-up and erythrocyte sedimentation rate (a measure of inflammation).

The antinuclear antibody (ANA) test detects autoantibodies that react against components of the nucleus, or "command center," of your own cells. A positive test indicates a stimulated immune system, which is common in people with lupus. However, ANA is also positive in people with other conditions, such as systemic sclerosis, dermatomyositis, undifferentiated and mixed connective tissue disease and rheumatoid arthritis. In addition, people without such disorders could have a positive ANA test. A positive ANA may also be found in people over 50.

Other autoantibody tests include anti-dsDNA, anti-Sm, anti-RNP, anti-Ro (SSA) and anti-La (SSB). Anticardiolipin or other antiphospholipid tests may indicate risk for a blood clotting disorder. In some cases, specialized diagnostic tests for the eyes, heart, lungs or brain, or a biopsy of the kidney may be performed.

Clearly, diagnosing lupus is a complex matter. If there is a reasonable likelihood that you have systemic lupus, you will need a referral to a rheumatologist.

Treatment

If diagnosed at an early stage, treated promptly and monitored routinely, systemic lupus erythematosus (SLE) is rarely fatal. You should make sure that every health care professional you deal with knows you have lupus.

Whether your disease is mild or severe, you have to stay under close medical supervision. You should let your health care professionals know immediately if you suffer any injuries, get sick or plan to become pregnant because many things may cause your disease to flare.

Typical warning signs of a flare are:

• increased fatigue, malaise and muscle aches (like the flu)
• a new or higher fever
• increased pain or swelling in the joints, especially when you wake up
• development or worsening of a rash, particularly one that is made worse by the sun
• shortness of breath or pain when breathing

Keep an open dialogue with a health care professional whom you trust and can easily reach in an emergency. If you are experiencing a flare, it might be important to receive diagnostic tests, change medications or postpone certain elective procedures or surgeries.

Although a lupus pregnancy is considered high risk, many women with lupus can carry their babies safely to the end of the term if they plan the pregnancy with the help of a rheumatologist and receive care from an experienced high-risk obstetrical team. Women with lupus have a higher rate of miscarriage and premature births compared to women without the disease. There are medications for lupus that are safe in pregnancy and important in controlling disease activity during pregnancy.

About 7 percent to 33 percent of women whose lupus has been in remission for at least six months prior to conception will have a lupus flare during pregnancy. In women with active lupus at conception, about 60 percent will experience a flare. Less than 20 percent experience miscarriage or death of a baby. About 20 percent of pregnant women with lupus will develop preeclampsia, a form of dangerously high blood pressure that increases the risk of miscarriage, low birth-weight babies, premature births and injury to the mother. Women with lupus also have increased risk of having preterm delivery or a baby with a low birth weight. Lupus outcomes are much better when lupus is under control before getting pregnancy.

Several over-the-counter and prescription medications are available to manage lupus symptoms including:

• Monoclonal antibody. Belimumab (Benlysta) is a monoclonal antibody, a laboratory-produced molecule that mimics the disease-fighting properties of the body's natural immune system. Approved by the U.S. Food and Drug Administration in 2011, it is the first new treatment for lupus in over half a century. Belimumab can be used to treat adults with active lupus who are also receiving standard therapy, such as corticosteroids, antimalarials, immunosuppressives and nonsteroidal anti-inflammatory drugs. Delivered intravenously, it targets B-lymphocyte stimulator (BLyS) protein, which may reduce the number of abnormal B cells thought to be a problem in lupus. Studies showed that people treated with belimumab, along with standard therapies, experienced less disease activity. The drug also may reduce likelihood of severe flares and allow some people with active lupus to reduce their steroid doses. The most common side effects in the studies were nausea, diarrhea, fever and reactions at the intravenous infusion site.
• Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen, as well as aspirin.
• Corticosteroids. These drugs, including prednisone (Deltasone), prednisolone (Prelone), hydrocortisone (Cortef) and methyl-prednisolone (Medrol), suppress the immune system and reduce inflammation. They can be taken orally, given through injection or used in cream formulations to treat skin lesions. However, they can lead to numerous side effects, including acne, weight gain, diabetes, cataracts, cardiovascular problems and heightened susceptibility to infections. People with lupus are especially prone to weakened or damaged bones, a side effect of high-dose or long-term corticosteroid treatment. People with lupus who take corticosteroids should talk to their health care professionals about taking vitamin D, calcium or other treatments to prevent osteoporosis.
• Antimalarial drugs, such as hydroxychloroquine sulfate (Plaquenil) and chloroquine (Aralen), can control a variety of lupus systems including arthritis, skin rashes, mouth ulcers, lung rashes, fatigue and fever. They promote healthy blood vessels and have an anti-inflammatory effect. Side effects of antimalarials include nausea or diarrhea and, in rare cases, damage to the retina of the eye. A person taking hydroxychloroquine treatment should be examined by an eye doctor every year to prevent unnecessary optical damage. Chloroquine requires more frequent monitoring. The beneficial effects of these treatments on symptoms are often slow at the beginning. It may take weeks or months for these medications to make an impact.
• Chemotherapy. Under some circumstances, chemotherapeutic drugs like methotrexate (Folex, Trexall, Rheumatrex), mycophenolate mofetil, azathioprine (Asasan, Imuran) and cyclophosphamide (Cytoxan) may be used to control symptoms of lupus. These are immunomodulating drugs, and they have some side effects, such as nausea, diarrhea, mouth ulcers, rashes, liver toxicity and bone marrow suppression, usually leading to low white blood cell counts. Cyclophosphamide, which may be used to suppress the immune system and inflammation, can be used safely, with care, but can have significant side effects, including gastrointestinal complications, hair loss and greater risk of infection. Methotrexate can cause inflammatory damage to the lung or liver.
• Other therapeutic agents. Drugs like cyclosporine, a strong immunosuppressive agent that is used primarily in organ transplant patients, may sometimes be used to control symptoms of lupus. There is a potential for kidney damage with cyclosporine.
• Anticoagulant medications. To help prevent blood clots, a serious complication of lupus, medications that help thin your blood—called anticoagulants—may be prescribed. These drugs include low-dose aspirin, heparin (Calciparine, Liquaemin), warfarin (Coumadin), and rivaroxaban (Xarelto). If you take certain anticoagulants, your health care professional will monitor you to make sure your blood doesn't become too thin.
• Topical steroids and nonsteroidal T cell inhibitors. Skin lesions can also be treated with topical steroids and topical nonsteroidal T cell inhibitors such as tacrolimus or pimecrolimus.

Prevention

While there is no way to prevent systemic lupus erythematosus (SLE), there are things you can do to reduce the risk and severity of lupus flares. There are also ways to reduce the potential side effects of medications used to treat the disease.

The first step is exercise. Getting low-impact, aerobic exercise regularly can reduce joint stiffness, lower stress levels and help prevent osteoporosis, weight gain and heart problems that may be caused by medications.

It is also important to determine which environmental factors may trigger your lupus flares. Triggers may include:

• Light. Exposure to the ultraviolet sunlight (and, to a lesser extent, fluorescent and halogen lights) can lead to a skin rash and worsen other lupus symptoms. It helps to wear protective clothing and use sunscreen when outdoors. It is best to use a sunscreen that protects from both UVA and UVB light. Also, remember that some UV light penetrates glass, including car windows. Try filters on windows to block UV light.
• Stress. People with lupus often link worsening symptoms or flares to stressful events such as divorce, job change or a death in the family. It is important to recognize that stress did not cause the immune imbalance of lupus and that it is OK to seek help when facing a chronic and sometimes disabling pattern of symptoms. Try not to underestimate the support you could get from family, friends, health professionals or workplace human resource services. It is equally important not to put your life on hold. Using common sense and getting plenty of rest and exercise is a good antidote to stress. Putting your life on hold and going to bed for the rest of your life is not.
• Overwork or lack of rest
• Infection
• Injuries or surgery
• Pregnancy and childbirth
• Suddenly halting lupus medications
• Sensitivities or allergies to items such as hair dye, hair permanent solution, makeup and skin creams
• Certain over-the-counter medications such as cough syrup or laxatives or immunostimulatory herbal medications
• Immunizations

Ongoing Research

There are many potential lupus treatments currently being studied in clinical trials that may become mainstream therapies in the future. Examples include drugs that target immunostimulatory cytokines, signaling pathways, receptors on cells that activate the immune system and cells that make autoantibodies, among many other targets. In addition, there are studies looking at stem cell transplants and at new medications for cutaneous lupus.

Facts to Know

1. Systemic lupus erythematosus (SLE) is a disease in which your body's immune system targets healthy tissue. Normally your immune system works by creating and dispatching antibodies, cells and special chemicals that react against antigens (molecules that lie on the surface of disease-causing viruses and bacteria). In lupus, antibodies attack normal tissue; potential targets for attack include the skin, joints, kidneys, brain, heart and lungs. In fact, this occurs in all healthy people to some extent. There is nothing bizarre about the autoantibodies or the inflammation of lupus; it is simply a matter of imbalance.
2. Approximately 1 million to 1.5 million Americans have some form of lupus. Women constitute at least 90 percent of systemic lupus cases. Lupus is more common in African-Americans, American Indians and Asians than in Caucasians.
3. There are several types of lupus that have specialized names: systemic lupus erythematosus (SLE), which can affect almost any organ system in the body; primary cutaneous lupus (the most common form is discoid lupus) involving mainly the skin; drug-induced systemic lupus erythematosus, a temporary reaction to certain drugs; and neonatal lupus, which occurs in babies born to mothers who had autoantibodies. A diagnosis of any of these types of lupus can be confusing because it can be serious and life threatening but is often mild to moderate in most people, especially with careful diagnosis and treatment.
4. The diversity of symptom type and severity can make lupus difficult to diagnose. Mild cases can go undiagnosed for years, and no single lab test can detect the disease. It is important to understand that a positive antinuclear antibodies (ANA) test alone does not give a person a diagnosis of lupus.
5. SLE symptoms tend to wax and wane. Patterns of inactive disease and lupus "flares" come and go. Lupus can be unpredictable; symptoms as well as the flare cycle may change at any time.
6. If you have a parent, child or sibling with lupus, your risk of developing the disease is somewhat increased, but it is not usually a very high risk and is not considered enough to warrant testing in the absence of symptoms. A positive ANA is common in family members of people with lupus and does not necessarily mean they will develop lupus.
7. Many women with lupus can have healthy babies with proper diagnosis and management.
8. Exposure to UV light can lead to a skin rash and worsen other lupus symptoms. If you have lupus, wear protective clothing and use sunscreen (SPF 55 or greater) when outdoors, and remember that some UV light penetrates glass, including car windows. Try to use sunscreen that protects against both UVA and UVB light. Studies indicate that sunscreens that contain ecamsule (Mexoryl) in addition to SPF block of 55 or higher can be particularly effective for photosensitive patients. Stronger sunscreens are required than for preventing skin cancer, because even small amounts of light can trigger a response in the skin in patients with lupus.
9. A chronic, unpredictable, waxing and waning illness is extraordinarily stressful. It may help to develop a plan for dealing with stressful situations that includes a support system of family and friends, health care professionals and community organizations. It is important to pace yourself, but it is equally important not to put your entire life on hold.
10. Lupus is not preventable or curable, but symptoms can be treated and flares minimized through medications and reasonable lifestyle modifications.

Questions to Ask

Review the following Questions to Ask about lupus so you're prepared to discuss this important health issue with your health care professional.

1. Do my symptoms suggest a possible diagnosis of lupus?
2. How much experience do you have diagnosing and treating lupus patients?
3. What tests should I undergo to determine whether lupus has caused any damage to my kidneys or other vital organs?
4. What lifestyle and dietary changes can I make to help me stay healthy?
5. What are the side effects or potential interactions of the drugs you are prescribing?
6. Can you recommend an appropriate exercise regimen?
7. What should I do if I decide to have a baby?
8. Can you refer me to a local support group for lupus patients?

Key Q&A

Lifestyle Tips

1. Thinking pink or blue? Plan your pregnancy
   Don't stop using birth control until your lupus has been stable in the mild to moderate range for at least six months. Identify an experienced, high-risk obstetrical team; a pediatrician experienced in caring for newborns from lupus pregnancies; and a hospital with the resources for specialized medical care during and after delivery, just in case.
2. Care for your skin
   To avoid the effects of sunlight as a trigger for skin flares or simply as source of long-term damage to skin, use sunscreen daily, through all seasons. Ask at the cosmetics counter for facial and body moisturizers with sunscreen SPF 30 to 50—many product lines have them. If possible, obtain a sunscreen that protects from UVA and UVB (contains ecamsule [Mexoryl]) and a high SPF of 55 or greater to screen UVB. Blend darker shades with your usual foundation to add contours and minimize "moon face" from steroid treatment. If you have skin lesions or scars that bother you, conceal them with makeup designed for the purpose.
3. Protect yourself from sunlight
   Though not all lupus patients experience sunlight as a trigger for skin flares, many do. Remember that windows don't block the ultraviolet (UV) light in sunlight, meaning that you can be exposed even when indoors or in a car or train, if you are near windows allowing light in. Fluorescent lights are also a source of UV light, but it may be possible to install filters. Use sunscreen year-round; look for SPF 55 or higher and protection against UVB rays, as well as UVA protection. Wear wide-brimmed hats and protective clothing with a tight weave, to block out sunlight.

Organizations and Support

For information and support on coping with Lupus, please see the recommended organizations and books listed below.

American College of Rheumatology
Website: http://www.rheumatology.org
Address: 1800 Century Place, Suite 250
Atlanta, GA 30345
Phone: 404-633-3777

Lupus Alliance of America
Website: https://www.facebook.com/Lupus-Alliance-of-America-Inc-107553585677/
Address: 3871 Harlem Rd.
Buffalo, NY 14215
Phone: 315-454-9886
Email: info@lupusalliance.org

Lupus Foundation of America
Website: http://www.lupus.org
Address: 2000 L Street, NW, Suite 710
Washington, DC 20036
Hotline: 1-800-558-0121
Phone: 202-349-1155
Email: info@lupus.org

Books

ABC of Asthma, Allergies and Lupus: Eradicate Asthma - Now!
by F. Batmanghelidj

Autoimmune Connection: Essential Information for Women on Diagnosis, Treatment, and Getting On with Your Life
by Rita Baron-Faust and Jill Buyon

Challenges of Lupus - Insights & Hope
by Henrietta Aladjem

Health Journeys For People With Rheumatoid Arthritis Or Lupus
by Belleruth Naparstek