Lupus

Definition

Lupus is an autoimmune disorder that may damage body tissues and lead to widespread, chronic inflammation and pain during periods of worsened symptoms called flares. Lupus can cause problems with multiple body systems and organs, including the joints, skin, kidneys, heart, lungs, and blood vessels. Although lupus cannot be cured, it is treatable with medications and other therapies.

Description

Lupus is an autoimmune disease. Normally, the white blood cells in the body's immune system protect a person from harmful substances called antigens.

Antigens may include bacteria, viruses , foreign blood, cancer cells, and other toxins that could cause disease or infection. To defend the body against antigens, the body produces antibodies. In a person with a healthy immune system, the antibodies then destroy the antigens, keeping the person from getting sick.

In people with autoimmune diseases, however, the immune system cannot tell the difference between an antigen and healthy tissues. As a result, the body begins attacking its own healthy tissues. In people with lupus, the autoimmune response most often attacks the joints, skin, heart, lungs, kidneys, and blood and circulatory system.

There are several different types of lupus, including: systemic lupus erythematosus (SLE); discoid lupus erythematosus (DLE); and drug-induced lupus. The severity of lupus symptoms vary from person to person: in some people, the symptoms may be mild and involve only the joints and skin; in others, the disease may be severe and cause joint, kidney, lung, heart, and bone complications.

Systemic lupus usually involves multiple organs and body systems and is usually more severe than the DLE form of the disease. The majority, about 70%, of all lupus cases involve this systemic form of the disease. People with SLE typically experience pain in the joints and muscles, fatigue, and skin rashes, which may come and go. These periods of more severe symptoms are called flares, whereas the milder periods of the disease are referred to as remission. People with SLE may experience kidney inflammation (nephritis), which can make it difficult for the body to remove toxins and other waste products. Lupus patients may be prone to develop pneumonia or inflammation of the chest cavity that makes it difficult to breathe. The disease may also contribute to central nervous system problems, including headaches, dizziness , seizures, behavior changes, and vision and memory difficulties. Having lupus also increases the risk of atherosclerosis (hardening of the arteries), blood clots , and deficiencies in red and white blood cells and platelets.

DLE, sometimes referred to as discoid or cutaneous lupus, primarily affects the skin and accounts for 10% of all lupus cases. People with this form of lupus typically develop a rash on the face, neck, and scalp but do not experience problems with the joints, kidneys, or heart. However, about 10% of people with DLE eventually develop SLE; doctors think that in these patients, the rash was an initial symptoms of systemic inflammation.

Drug-induced lupus may also develop after a person takes certain prescription medications. People with this type of lupus tend to have symptoms similar to those of SLE, but the symptoms of lupus typically fade within days, weeks, or months of discontinuing the medications. Medications that may induce lupus include hydralazine (a drug used to treat high blood pressure ) and procainamide (a drug used to treat irregular heart rhythms). About 4% of people who take these medications develop drug-induced lupus.

Demographics

Lupus affects people of all ages and races, but it is most common in women. Ninety percent of the 1.4 million diagnoses of lupus in the United States are made in women. Lupus is also more prevalent among younger women between 15 and 44 and women of particular ethnic groups. Hispanic/Latino, African American, and American Indian women are more likely to develop lupus than white women, and their symptoms tend to be more severe.

However, according to the Lupus Foundation of America, 15% of people with SLE develop it later in life—after age 55. Late onset lupus affects women eight times more than men, and is more prevalent in Caucasians, although it can occur in people of any race.

Drug-induced lupus is also more common in older adults because of the greater likelihood that they have other conditions (such as heart disease and high blood pressure) that require medication. When a person goes off the medication, the symptoms of drug-induced lupus eventually go away.

Causes and symptoms

The causes of lupus are unknown, although physicians have several clues about what might trigger this autoimmune disease in some people. Because lupus often runs in families, researchers think there is a genetic component to the disease. However, other factors, including environment, stress , the use of certain medications, and exposure to sunlight, may also influence lupus development and exacerbate flares.

Symptoms of lupus depend on the type a person has and can vary widely. Some common symptoms of lupus include:

• a signature red rash or color change in the skin across the nose and cheeks (this is also called a malar rash; often it is in the shape of a butterfly)
• painful, swollen joints (arthritis) and glands
• fevers that can't be explained by illness
• pain in the chest when breathing
• extreme fatigue
• anemia (loss of red blood cells)
• hair loss
• sensitivity to the sun
• blood flow problems in the fingers when cold or stressed
• depression
• problems with memory or thinking clearly

For some people, mouth sores, seizures, hallucinations, and kidney problems signal lupus.

In people with late-onset lupus, symptoms tend to be milder and include arthritis, pleurisy, pericarditis , dry eyes and mouth, and muscle aches. In older adults, it may be harder to diagnose lupus because the symptoms mimic other diseases common in this age group, such as rheumatoid arthritis .

Lupus symptoms may come and go. These periods of worsened symptoms, called flares, may be triggered by spending time in the sun or during a time of emotional stress.

SLE has also been linked to a higher risk of developing osteoporosis , a disease that makes bones brittle and more likely to break. Osteoporosis may occur in lupus patients because the steroid medications often prescribed to reduce inflammation can lead to bone loss. Fatigue and pain in the joints and muscles also makes it more likely a person will remain inactive, which increases the likelihood of bone loss. Finally, lupus itself may contribute to weakened bones that are more likely to break.

According to the National Institute of Arthritis and Musculoskeletal and Skin Diseases, women with lupus may have more than five times the risk of a bone fracture from osteoporosis.

Diagnosis

Obtaining a lupus diagnosis can be difficult for several reasons: the symptoms of lupus flare and disappear over long periods of time; the severity and type of lupus symptoms can vary widely from person to person; and finally, there is no one test that can diagnose the disease. Instead, doctors must rely on several diagnostic techniques to confirm a lupus diagnosis, including a detailed medical history, physical examination, blood and urine tests, and skin or kidney biopsies.

When conducting a medical history, doctors may ask patients a variety of questions, such as:

• Have you had stiff, tender, and swollen joints? Is this worse in the morning?
• Do you ever feel extremely tired for days or weeks, even when you're getting plenty of sleep at night?
• Have you ever felt pain in your chest when taking deep breaths?
• Does your skin break out when you're in the sun, but not from sunburn?
• Have you had a rash across your nose and cheeks? Is it in the shape of a butterfly?

In addition to taking a thorough medical history, doctors will also conduct a physical examination. A physician may listen to the heart (in some lupus patients, doctors can hear a sound called a heart friction rub) and conduct a neurological exam. Typically, a person must have four out of 11 typical symptoms of the disease (as defined by the American College of Rheumatology) before a diagnosis can be confirmed.

Laboratory tests are also an integral part of the lupus diagnosis process. One test, called the antinuclear antibody (ANA) test, is often checked when a doctor suspects a person has lupus. In this test, a person's blood is checked for autoantibodies that are often present in the blood of people with lupus. Testing positive for ANA does not automatically mean a person has lupus, but it can help doctors make a diagnosis when considered with a person's physical symptoms. Other tests doctors may use to confirm a lupus diagnosis include the anti-double strand DNA (dsDNA), anti-Smith antibodies (Sm), sedimentation rate (ESR), and C-reactive protein binding.

If lupus is diagnosed, doctors may check a person's urine for signs of kidney problems, order chest x-rays for signs of inflammation in the lungs or heart, and have the patient's blood checked for problems with the white blood cells to see how far the disease has progressed.

Treatment

Several types of health care professionals may work together to treat an older adult with lupus. Family doctors or internists, rheumatologists (specialists in rheumatic diseases), immunologists (specialists in immune system disorders), and other specialists may play a role in treating the lupus patient.

The treatment a person receives for lupus depends on the type of lupus and the extent and severity of the disease. Both over-the-counter and prescription medications may be recommended, such as:

• Nonsteroidal anti-inflammatory drugs (NSAIDS):These drugs, which include ibuprofen and naproxen, reduce inflammation and control pain, swelling, and fever. However, these medicines also may cause side effects such as nausea, heartburn, and diarrhea as well as liver, kidney, and neurological complications with prolonged use, so it's important that a person taking these drugs for lupus does so under the direction of a doctor.
• Antimalarial drugs: Antimalarial drugs such as hydroxychloroquine treat the fatigue, joint pain, rashes, and lung inflammation caused by lupus and may prevent flares from occurring. Side effects include nausea and, in rare cases, vision problems.
• Corticosteroids: A variety of corticosteroid medications, including prednisone, hydrocortisone, methyl-prednisolone, and dexamethasone, can suppress the inflammation often associated with lupus. Lupus patients take these drugs in pill form, apply creams to the skin, or receive corticosteroid injections. Despite their effectiveness, corticosteroid drugs do have short-term side effects, such as increased appetite and weight gain. Long-term side effects may include high blood pressure, weakened bones, artery damage, diabetes, and cataracts.
• Immunosuppressive agents: These drugs, including cyclophosphamide and mycophenolate mofetil, block the production of immune cells and are typically used in lupus patients who experience kidney or central nervous system problems. These drugs. A person taking immunosuppressives may experience nausea and vomiting, as well as bladder problems, hair loss, decreased fertility, and an increased risk of infection.

Doctors may also use arthritis drugs to help control symptoms of lupus and reduce the risk of flares.

Nutrition/Dietetic concerns

There are no specific guidelines for people with lupus to follow, however, there are several nutritional considerations that may be impacted by a lupus diagnosis.

Lack of appetite and weight loss is common among people who have recently been diagnosed with lupus. Appetite and weight loss may be related to a person's symptoms of pain and fatigue, or they may be a side effect of common lupus medications. In addition, some people develop mouth sores when taking lupus medications, another factor that can make it difficult to eat. Older adults struggling with a lack of appetite should talk to their health care providers, who may recommend consulting with a registered dietitian who can suggest a diet that works best with the patient's needs and lifestyle.

Weight gain is also a common side effect of corticosteroids, drugs that are often used to treat the inflammation of lupus. If a person has gained weight after starting lupus medications, a registered dietitian (RD) can also help by devising a meal plan that incorporates nutritious foods that won't add excess weight. A RD can also help lupus patients work toward controlling high blood pressure and avoiding atherosclerosis. These cardiovascular complications may be more common in lupus patients, but a combination of a low-fat diet and exercise may reduce the risk of these common complications.

Also, lupus patients taking corticosteroids may need to take vitamin D and calcium supplements to counteract the bone-damaging effects of the disease and reduce the risk of osteoporosis.

In general, if medication use or lupus symptoms are making it difficult to eat, patients should consult with a doctor or nurse, who can provide additional information.

Therapy

Medications for lupus are costly, and many have the potential for serious adverse side effects. As a result, some patients turn to other therapies to relieve lupus symptoms.

Massage and acupuncture are just a few of the alternative and complementary therapies that may be used by lupus patients. Doctors may encourage lupus patients to get regular, gentle exercise during remission to increase joint flexibility and muscle strength.

In addition, some patients have tried dietary supplements in an attempt to alleviate lupus symptoms. Supplementation with omega-3 fatty acids found in fish oils could hold promise for lupus patients. In one study of 60 people with SLE, daily doses of 3 grams of omega-3 fatty acids in the form of fish oil supplements over a 6-month period improved lupus symptoms. Not only did the supplements appear to relieve joint pain, but they also improved blood vessel function, researchers noted.

Prognosis

Currently, there is no cure for lupus. The good news for older adults with this disease is that there are medications to reduce the severity and discomfort of many lupus symptoms. Some people with lupus do need to be hospitalized if they experience multi-system organ failure, however, the Lupus Foundation of America asserts that most people with lupus rarely require hospitalization.

Some people with lupus do die from the disease, although it is not common. The Centers for Disease Control and Prevention estimated that more than 1,000 people die from lupus annually, and older adults, women, and blacks had the highest death rates among lupus patients.

Following the doctor's instructions, taking medications exactly as they're prescribed, and getting help when symptoms flare can help lupus patients extend the quantity and quality of their lives.

Prevention

Unfortunately, doctors have not yet discovered how to prevent the development of lupus. However, preventing lupus flares from occurring or getting worse is possible.

To prevent flares, older adults should learn to identify their own personal warning signs that a flare is about to occur and talk to their doctor if they suspect a flare is imminent. In addition, getting regular health care and laboratory tests can help doctors note changes and make adjustments once a flare begins. Finally, because they are at risk for other complications from lupus, older adults should have their blood pressure and cholesterol checked regularly. An annual influenza vaccine may also be recommended, and patients should reduce exposure to the sun and always wear sunscreen.

Other strategies, such as reducing stress, getting regular exercise, and establishing a good support system may help people with lupus stay healthy long-term.

Caregiver concerns

Care givers can help by learning the signs of their loved ones' flares and encouraging communication with the doctor when lupus symptoms occur.

Care givers should keep in mind that uncontrolled bleeding, trouble breathing, fainting , confusion, chest pain, or seizures in a lupus patient are signs of a serious problem. These symptoms indicate their loved one with lupus needs immediate medical help.

QUESTIONS TO ASK YOUR DOCTOR

• What is the best way to treat my lupus symptoms?
• Is there anything I need to avoid to reduce the risk of flares?
• Does having lupus put me at risk for other health conditions?
• Will the prescribed medications cause dangerous interactions with other medicines I take?
• I take herbal or vitamin supplements. Is there anything I need to know?
• Are there exercises you can recommend to help me cope with my lupus and reduce the risk of osteoporosis?
• Can you recommend any support groups for me and my family?

KEY TERMS

Antinuclear antibody (ANA) test —A test often used to look for autoantibodies that react against components of the nucleus of the body's cells. Many people with lupus test positive for ANA.

Arthritis —A condition characterized by inflamed, swollen, painful joints.

Autoimmune disease —A disease in which the immune system makes antibodies that mistakenly attack the body's healthy organs and tissues.

Nephritis —Inflammation of the kidneys.

Osteoporosis —A condition that makes bones less dense and more likely to fracture.

Pericarditis —Inflammation of the sac around the heart.

Pleurisy —Chest pain that occurs when a person takes a deep breath.

Resources

BOOKS

Wallace, Daniel. The Lupus Book: A Guide for Patients and Their Families. Third Edition. Oxford University Press, 2005.

PERIODICALS

Wright SA, O'prey FM, et al. A randomised placebo-controlled interventional trial of omega-3-polyunsaturated fatty acids on endothelial function and disease activity in systemic lupus erythematosus. Annals of the Rheumatic Diseases, 2007 Sep 17, epub.

ORGANIZATIONS

Alliance for Lupus Research, 28 West 44th Street, Suite 501, New York, NY, 10036, 212-218-2840, 800-867-1743, [email protected], http://www.lupusresearch.org/home.html.

Lupus Foundation of America, 2000 L Street, N.W., Suite 710, Washington, DC, 20036, 202-349-1155, 800-558 0121, 202-349-1156, http://www.lupus.org/newsite/index.html.

National Institute of Arthritis and Musculoskeletal and Skin Diseases, 1 AMS Circle, Bethesda, MD, 20892-3675, 301-495-4484, 877-226-4267, 301-718-6366, [email protected], http://www.niams.nih.gov.

S.L.E. Lupus Foundation, 330 Seventh Avenue, Suite 1701, New York, NY, 10001, 212-685-4118, 212-545-1843, [email protected], http://www.lupusny.org.

Amy Sutton