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, info@lupusresearch.org, 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, NIAMSinfo@mail.nih.gov, 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, Lupus@LupusNY.org, http://www.lupusny.org.

Amy Sutton

Lupus

Definition

Lupus, also known as lupus erythematosus, is an autoimmune inflammatory disorder that occurs mostly in women.

Description

Lupus produces widely varying symptoms, although joint pain is reported by most patients and skin lesions are common. Lupus can cause short periods of symptoms alternating with healthy periods, or can progress into a life-threatening disorder affecting the heart, kidneys, and other organs.

Why the disease is termed lupus is unknown, but it has been known as a distinct disorder and called lupus by European physicians since at least the tenth century a.d. The term erythematosus was first attached to the disease in the 1850s, and it refers to the patchy congestion of skin capillaries with blood (erythema) that often accompanies the disease.

Demographics

Between one million and 1.5 million Americans have some form of lupus. The incidence among women is 10–15 times greater than among men, and it is two to three times more common among African Americans, Hispanics, Asians, and Native Americans than among whites. Lupus most often appears for the first time in women between the ages of 15 and 44. Twenty thousand people die of lupus-related causes in the United States annually.

Causes and symptoms

Lupus is an autoimmune disorder, a disease in which the body's immune system turns against the body itself. In a healthy person, the immune system defends against invading organisms but does not, in general, attack the body's own tissues. The cause of lupus is unknown. However, it is known that lupus has a genetic component, which means a predisposition to lupus can be inherited. Approximately 10% of lupus patients have one or more direct relatives with lupus. (Note that this means that 90% of lupus patients have no such relatives; however, it shows a

genetic connection because 10% is a much higher figure for familial lupus than can be attributed to chance alone.) Lupus has been definitely linked to genes on chromosome 1 and less certainly to genes on chromosomes 4 and 6.

Given genetic susceptibility, the disease may either develop spontaneously or be triggered by some environmental factor. Environmental factors known to trigger lupus include infections (e.g., Epstein-Barr virus, which infects 99% of children with lupus, but only 70% of healthy children), antibiotics, ultraviolet light (the rays in sunlight or sunlamp-light that causes sunburn), stress, smoking, certain medications, and hormones (especially estrogen, the female sex hormone).

Lupus manifests as a continuum or spectrum of disorders. However, it is common to divide lupus cases into four categories or groups:

• Systemic lupus erythematosus. This is the most serious form of lupus and affects about 70% of all persons with lupus. It is termed systemic because, in this variety of lupus, the body's immune system attacks one or more essential body systems. Targets may include the brain, kidneys, heart, pancreas, or other organs.
• Discoid or cutaneous lupus erythematosus. This variety of lupus is less severe, in that it attacks the skin only. However, it can be disfiguring, often attacking the skin of the face. The term discoid is derived from the round (disc-shaped) lesions that appear on the skin. About 10–15% of lupus patients have cutaneous lupus.
• Drug-induced or drug-related lupus erythematosus. This term refers to lupus that develops after a patient has taken a medication. Medications that can trigger drug-induced lupus include procainamide or hydralazine. Many of the substances that can potentially trigger lupus fall into the class of aromatic amines, or hydrazines. For example, the aromatic amine paraphenylenediamine is present in certain hair dyes and has been associated with lupus or lupus-like syndrome. Tartrazine (a food coloring, FD&C yellow No. 5), which is present in thousands of foods and medications, has also been associated with lupus. Cocaine abuse can induce lupus and several other connective-tissue diseases, as can exposure to certain metals (e.g., mercury). Between 10,000 and 15,000 people are diagnosed with drug-induced lupus annually in the United States.
• Mixed connective tissue disease. Approximately 10% of patients with lupus also have symptoms of one or more additional connective-tissue diseases.

The symptoms of lupus are quite varied. In discoid lupus, red patches (erythema) appear symmetrically on the cheeks, possibly extending to the face, neck, scalp, and other parts of the body. No organ other than the skin is affected (or the disease is classified as systemic, rather than discoid). Systemic lupus may begin suddenly, signaled by fever, or develop slowly over months or years. Chronic fatigue is a common symptom. Symptoms related to impairment of any organ may occur. The lupus disease process in a given organ is named after that organ; for example, inflammation of the kidneys is termed lupus nephritis, and inflammation of the brain is termed lupus cerebritis. Kidney involvement may be fatal. Over 50% of all systemic lupus patients in the United States presently have some degree of lupus cerebritis; 25–75% have neuropsychiatric symptoms at some time in their illness. Symptoms of lupus cerebritis may include headaches, seizures , stroke , psychosis, dementia , peripheral neuropathy , cerebellar ataxia (failure of muscular coordination, usually on one side of the body), chorea (jerky, involuntary movements), and others. Duration of central nervous system involvement may be transient (as with a migraine headache ) or long lasting (as with dementia). Stroke incidence is 3–20% in systemic lupus patients, and is highest in the first five years of the disease. Peripheral neuropathy (carpal tunnel syndrome , for example) occurs in more than 20% of systemic lupus patients and cranial nerve palsies occur in 10–15%.

Exposure to the ultraviolet rays in sunlight can trigger lupus or, in a person who already has the disease, cause it to flare up. Worsening flare-ups of the disease can be life threatening because they can include inflammation and failure of the kidneys. Also, declining memory and mental sharpness with long-term lupus is common.

Diagnosis

Lupus is notoriously difficult to diagnose. Many cases are not diagnosed until the patient has suffered irreversible kidney damage; for patients who do not have organ-threatening disease, diagnosis takes an average of two years of searching among physicians and conditions. The telltale erythematous skin lumps or rashes that give lupus erythematosus the latter half of its name eventually appear in 90% of systemic lupus patients and all discoid lupus patients, but may not appear early enough in the course of the disease to guarantee timely diagnosis. Additionally, no single lab test can confirm lupus, although certain antibody tests can help to distinguish lupus from other diseases.

Diagnosis of systemic lupus is based on a list of 11 criteria listed by the American College of Rheumatology. If four or more of the 11 criteria are met, a patient is deemed to have systemic lupus. The criteria include discoid or macular rash (often in a classic facial butterfly pattern across the nose and cheeks), photosensitivity, ulcers in the mouth, kidney dysfunction, and the presence of various blood factors such as anti-DNA antibody or anti-nuclear antibody (antibody that targets cell nuclei).

Approximately 15% of diagnoses of lupus may be misdiagnoses of other disorders, including fibromyalgia, seronegative spondyloarthropathies such as ankylosing spondylitis or Reiter's syndrome, autoimmune thyroiditis, and multiple sclerosis .

Although diagnosis of lupus cerebritis is particularly difficult, even if a patient has lupus, this does not necessarily mean that the neurological symptoms are due to lupus. Imaging studies cannot necessarily distinguish lupus cerebritis, although magnetic resonance imaging (MRI) studies are considered helpful. Positron emission tomography (PET) imaging has a high sensitivity to changes in the brain resulting from lupus cerebritis.

Treatment team

As with other neurological diseases in which the spectrum of symptoms varies widely, the treatment team must be designed for each individual case of lupus. A dermatologist will be involved if skin lesions are present; a neurologist , if cognitive loss is a possibility; a nephrologist will monitor kidney function; and a rheumatologist is often involved because of the frequency of joint pain. Other specialists will be needed depending on what organ systems are affected.

Treatment

There is no known cure for lupus. However, there are numerous interventions designed to lessen the severity of the disease. These interventions can be classed as pharmacologic (drug-based) or nonpharmacologic.

Pharmacologic interventions (drug therapies)

Five categories of medication are used to treat systemic lupus patients: sunscreens and steroid lotions, nonsteroidal anti-inflammatory drugs (NSAIDs, e.g., acetaminophen or ibuprofen), corticosteroids (e.g., prednisone to suppress the autoimmune response and control inflammation), anti-malarial drugs, and cytotoxic agents (i.e., chemotherapy drugs that are used for cancer, such as methotrexate, azathioprine, and cyclophosphamide).

Cytotoxic agents are used in order to decrease steroid dosage. Anticoagulants (blood thinners) may also be prescribed. For patients with non-organ-threatening disease, the antimalarial drug hydroxychloroquine is often prescribed; prednisone is often prescribed in cases of organ-threatening disease. New lupus drugs are under investigation; with recent increases in knowledge about the genetic and molecular basis of autoimmune disorders, including lupus, pharmacological treatment breakthroughs are possible at any time.

Nonpharmacologic (non-drug) interventions

All persons with lupus should guard against exposure to the sun and use protective clothing, sunscreen, and common sense when going outdoors. Adequate exercise can protect against fatigue, obesity, osteoporosis (weakening of the bones), and hyperlipidemia (excessive fats in the blood plasma). In some cases, dietary restrictions may be helpful, including especially the avoidance of food allergens and foods that may trigger lupus symptoms (such as alfalfa seeds). Vitamins, minerals, and dietary fatty acids have been shown to moderate lupus symptoms in some cases. On the other hand, some dietary supplements such as melatonin and Echinacea can worsen symptoms of some autoimmune diseases.

For lupus cerebritis, therapy choices include all the above options for alleviating the disorder throughout the rest of the body. Drug therapy can also include psychotropic medications such as antipsychotics, antidepressants, and benzodiazepines to stabilize mood, if this is affected. Unfortunately, long-term use of corticosteroids, one of the mainstays of pharmacological lupus treatment, may itself cause psychiatric symptoms. Experimental investigation of pheresis of cerebrospinal fluid for treatment of lupus cerebritis (cerebrospinal fluid is withdrawn from, filtered, and returned to the patient) was begun in the early 1990s.

Clinical trials

As of mid-2004, approximately 25 lupus-related clinical trials were in progress, including investigations of monoclonal antibody therapy, the genetics of lupus, quality-of-life improvement, ultraviolet light therapy, stem-cell transplantation therapy, the mechanisms of kidney and brain damage, and many other aspects of lupus. Updated information on these trials can be found at the National Institutes of Health clinical trials website at <http://www.clinicaltrials.gov> for up-to-date information.

Prognosis

Prognosis for the individual patient depends on the severity of the disease process. Lupus can be fully compatible with a normal lifespan, or can result in fatal organ failure, depending upon the progression of the disorder in each individual.

Before corticosteroids became available, half of all patients with systemic lupus died within two years. Today, half of systemic lupus patients with organ-threatening complications survive for 20 years or longer. However, most systemic lupus patients eventually die from infections or from heart disease complicated by long-term use of corticosteroids.

There is some evidence that lupus may spontaneously resolve in part or whole, or resolve in response to treatment, in some lupus patients who have had the disease long term (i.e., 10 years or more).

Special concerns

Psychological counseling may be helpful, given that a diagnosis of lupus is life altering, and stress and frustration can enhance symptoms while searching for a diagnosis. Genetic counseling may be appropriate, as children of women with lupus have a 10% chance of developing lupus if female and 2% if male, while 20% of offspring overall will develop an autoimmune disorder of some type.

Resources

BOOKS

Phillips, Robert H., et al. Coping with Lupus: A Practical Guide to Alleviating the Challenges of Systemic Lupus Erythematosus, 3rd ed. New York: Avery Penguin Putnam, 2001.

Wallace, Daniel J. The Lupus Book: A Guide for Patients and Their Families. New York: Oxford Press, 2000.

PERIODICALS

Marshall, Eliot. "Lupus: Mysterious Disease Holds Its Secrets Tight." Science (April 26, 2002).

Nickens, Candice. "Treating Systemic Lupus Erythmatosus." Minority Health Today (July 1, 2000).

Rushing, Jill D. "Managing Organ-threatening Systemic Lupus Erythematosus." MedSurg Nursing (December 1, 2003).

"Systemic Lupus Erythematosus: Guidelines for Control." Consultant (February 1, 2000).

OTHER

"NINDS Neurological Sequelae Of Lupus Information Page." National Institute of Neurological Disorders and Stroke. April 24, 2004 (June 1, 2004). <http://www.ninds.nih.gov/health_and_medical/disorders/lupus_doc.htm>.

ORGANIZATIONS

Lupus Foundation of America. 2000 L Street, N.W., Suite 710, Washington, DC 20036. (202) 349-1155; Fax: (202) 349-1156. <http://www.lupus.org/>.

Larry Gilman

Lupus

What Is Lupus?

Who Gets Lupus?

What Causes Lupus?

What Are the Symptoms of Lupus?

How Is Lupus Diagnosed?

How Is Lupus Treated?

Is There α Cure for Lupus?

Living with Lupus

Resources

Lupus (LOO-pus) is a chronic (long-lasting) disease that causes inflammation of connective tissue, the material that holds in place the various structures of the body.

KEYWORDS

for searching the Internet and other reference sources

Autoimmune disorders

Rheumatology

“Oh, not again!” shouted 18-year-old Julia, her toothbrush falling into the sink with a clatter. It was the fifth morning this week that it had slipped from her hands. She had been waking up with achy, puffy hands, and she was finding it hard to grip things with her stiff fingers. A few weeks later, when filling out a form for a back-to-school physical, she wrote about her stiff hands and also mentioned that she had been feeling really tired all summer. Her doctor suggested she get some blood tests and called Julia’s mother a few days later with a referral to a rheumatologist*. He suspected that Julia had lupus.

* rheumatologist

(roo-ma-TOL-o-jist) is a doctor who specializes in disorders involving the connective tissue structures of the body.

What Is Lupus?

Lupus causes inflammation of connective tissues in the body. Connective tissue is the material that holds the various structures of the body in place. The cause or causes of lupus are uncertain. However, it is believed to be an autoimmune disorder, which is a reaction of the immune system against one’s own body. The medical name for lupus is lupus erythematosus (er-i-thee-ma-TO-sus).

There are two main types of lupus. These are discoid lupus erythematosus (DLE), which mostly causes reddened patches on the skin, and systemic lupus erythematosus (SLE), which affects the skin but also involves other tissues and organs.

A third condition, called lupus vulgaris, is unrelated to lupus erythematosus. It is a rare form of tuberculosis that typically produces nodules, or small lumps, on the skin.

Who Gets Lupus?

Lupus is a disorder that occurs in people of all ages worldwide. In the United States, it has been estimated that there are half a million lupus patients, or about one person in every 600.

Although members of both sexes can get lupus, the great majority of people affected by the disease are women (at least 9 out of 10 in the case of SLE, and approximately 7 out of 10 in the case of DLE). The majority of women affected are young to middle-aged. Like Julia, about half of SLE patients show their first signs of the disorder between the ages of 15 and 25.

In the United States, lupus is about three times more prevalent in people of African ancestry than in people of European ancestry. It also appears to be more prevalent in people of American Indian and Asian origin.

What Causes Lupus?

Lupus is not a contagious disease. One person cannot catch it from another, and there is no need to avoid being near someone who has lupus.

In about 10 percent of cases, certain prescription drugs, such as those used for irregular heartbeat or high blood pressure, can cause symptoms of SLE. (The symptoms usually go away when the drugs are discontinued.) This effect is noted most often in elderly patients and is referred to as drug-induced lupus. In the other 90 percent of SLE cases and in all instances of DLE, the cause or causes of the disorder are not known with certainty.

Although most people who have studied lupus agree that it is an autoimmune disorder, this knowledge provides only a part of the explanation of its cause. The reason is that the causes of immune disorders are not themselves very well understood. There are, however, some things that doctors believe can play a role in the development of lupus.

Heredity is assumed to play a part, although it is not known exactly what its role is. It has been determined that a person with a close relative who has SLE is slightly more likely to develop the disease (by about 10 percent) than someone who does not. The likelihood increases if one has an identical twin with SLE.

Its higher prevalence in some racial groups also suggests that heredity plays a role. However, no gene (hereditary factor) has been specifically linked to the development of lupus.

The fact that women are much more likely to get lupus than men suggests that hormones also may be associated with the disorder.

Certain environmental influences are believed to play a part in the onset of lupus. It is believed that autoimmune responses in some individuals may be triggered by bacteria, viruses, extreme stress, sunlight, certain antibiotics, or food additives. But not everyone gets lupus because they caught a particular virus or bacteria, are stressed about something in their lives, took a certain antibiotic, or ate a certain food.

The antibodies involved in the autoimmune response are called “autoantibodies.” They are thought to react specifically with certain chemical constituents of the cells in the body, producing inflammation and damage to organs and tissues.

Did You Know?

• The name “lupus erythematosus” comes from lupus, which is Latin for “wolf,” and “erythema,” which refers to reddened skin. In the past, people thought that patients with the facial rash looked as though they had been bitten or scratched by a wolf.
• The large majority of lupus patients are women.
• Lupus is not contagious.
• There is no one symptom or test that means someone has lupus.
• Many lupus patients develop arthritis.
• Many people with lupus have to stay out of the sun.
• Most lupus patients can lead nearly normal lives.

What Are the Symptoms of Lupus?

The signs and symptoms of lupus vary greatly in different individuals, both in the parts of the body involved and the degree of severity. The symptoms may also come and go, and may go away for weeks or months. Although lupus occurs much more frequently in women than in men, the symptoms in males are no less severe than in females.

Discoid Lupus Erythematosus

The mildest and most common form of lupus, DLE usually involves only the skin. It produces a rash of thickened, scaly reddish patches on the face and sometimes other parts of the body. Often the rash spreads in a characteristic butterfly-shaped pattern over the cheeks and bridge of the nose. After a few weeks or months, when healing has taken place, dark-colored or pale scars may remain. If the condition extends to the scalp, the person may lose some of his or her hair.

Sunlight tends to trigger and worsen the rash of lupus. In DLE, the patches sometimes nearly disappear during the winter months. Most people who have DLE are otherwise in good health. The condition only rarely progresses to SLE.

Systemic Lupus Erythematosus

People with SLE may have the same type of rash as those with DLE, and they may lose hair as well. In SLE, however, the skin lesions may spread and cause damage to the mucous membranes and other tissues. In some SLE patients the skin is not affected.

Arthritis may be the first symptom in some people with SLE. In fact, SLE is medically classified in the same family of diseases as rheumatoid arthritis, a disorder that causes painful inflammation in the joints. Other early symptoms may include weakness, extreme fatigue, fever, sensitivity to sunlight, and loss of weight.

Internal organs also may be affected by SLE, often causing serious disorders. Problems with kidney function are common in SLE patients, and uremia (yoo-REE-me-a), or the buildup of toxic substances in the blood due to kidney failure, can be fatal. The nervous system may be affected, causing psychological problems, seizures, or other symptoms. The lungs, heart, liver, and blood cells may also be involved.

In the blood, the presence of certain antibodies called anti-phospholipid (AN-ti-fos-fo-LIP-id) antibodies interferes with the normal function of the blood vessels, and can bring on a stroke or heart attack. In pregnant women, the presence of these antibodies can cause a miscarriage.

How Is Lupus Diagnosed?

Lupus can be hard to diagnose, especially if there are just a few symptoms. There is no one sign or symptom that definitely means someone has lupus, and there is no single laboratory test that will diagnose it either. For this reason, a combination of observations and tests is always necessary.

What Is an Autoimmune Disorder?

Nearly everyone is familiar with allergies such as hay fever and asthma, either in their own experience or that of friends or family. An autoimmune disorder might best be understood as a kind of allergy, except that the immune system attacks parts of one’s own body instead of outside substances such as dust and pollen.

The normal function of the immune system is to protect the body from invading microorganisms or toxic substances. In order to perform this function, it produces antibodies and special white blood cells (lymphocytes) that will recognize and destroy the intruders.

In autoimmune disorders, these responses (for reasons that are as yet not fully understood) occur against the body’s own cells, tissues, and organs. This reaction can produce a number of illnesses, including rheumatoid arthritis, a type of diabetes, and lupus.

Diagnostic tests for SLE include blood tests for certain antibodies that attack the nucleus of cells, and for LE cells. LE (lupus erythematosus) cells are white blood cells that destroy other blood cells and are an indication of lupus. Sometimes a skin biopsy (removal of a small sample) is performed to examine for antibodies active in lupus.

Early diagnosis is important so that treatment can be started as soon as possible.

How Is Lupus Treated?

Various types of treatment may be chosen depending upon the particular needs and symptoms of lupus patients. A variety of drugs are prescribed to counteract pain, inflammation, and related problems.

NSAIDs (nonsteroidal anti-inflammatory drugs), such as aspirin, ibuprofen, and naproxen, are used to lessen pain and inflammation in the joints and muscles. Another group of medications, called corticosteroids (kor-ti-ko-STEER-oids), is prescribed to reduce inflammation and activity of the immune system. A third group, called anti-malarials because they also are used to treat malaria, is often prescribed for skin and joint symptoms. Skin eruptions are treated with ointments and creams that contain corticosteroids and sunscreens.

Drugs prescribed to treat lupus can often cause unwanted side effects. For this reason, and because symptoms may change, ongoing medical advice with regular checkups is needed.

Is There α Cure for Lupus?

There is no cure for lupus, but effective treatment can maintain normal body function and control symptoms in the great majority of patients. Nonetheless, SLE can be a life-threatening disease, particularly when the kidneys are involved. The most common causes of death are kidney failure, bacterial infection, and heart failure.

Living with Lupus

There is no specific preventive measure, like vaccination, that one can take to keep from getting lupus. If one is diagnosed with lupus, however, there are ways to reduce the likelihood of “flares,” or sudden worsening of symptoms. Patients who are sensitive to sunlight can help prevent rashes by avoiding excessive exposure to the sun, using sunscreens, or wearing broad-brimmed hats. Although there is no vaccination for lupus itself, immunization against other infections is recommended.

Lifestyle changes can help patients with lupus avoid or control symptoms as well. Regular exercise can prevent some muscle weakness and fatigue. Smoking and excessive alcohol intake can be harmful, as they are to anyone. Changing such negative habits can bring about general improvement in one’s health. Joining support groups and talking with family, friends, and physicians can ease the effects of stress.

See also

Arthritis

Kidney Disease

Resources

Lahita, Robert G., and Robert H. Phillips. Lupus: Everything You Need to Know. Garden City, NY: Avery Publishing Group, 1998. A nontechnical guide written in a question-and-answer format.

Blau, Sheldon Paul, and Dodi Schultz. Living with Lupus: All the Knowledge You Need to Help Yourself. Reading, MA: Perseus Books, 1993. A comprehensive guide to coping with lupus medically and in daily life.

The National Institutes of Health posts information about lupus on its website. http://www.nih.gov/niams/healthinfo/slehandout

Further information may be obtained from the website of the Lupus Foundation of America. http://internet-plaza.net/lupus/

LUPUS

DEFINITION

Lupus is a disease in which a person's immune system attacks the body's own organs and tissues (see autoimmune disorders entry). The complete medical name for lupus is systemic lupus erythematosus (SLE; pronounced LOO-puhs er-uh-THEM-uh-tuhs).

DESCRIPTION

The immune system is a network of cells and tissues that protect the body against foreign organisms, such as bacteria and viruses. One mechanism used by the immune system is the release of antibodies. Antibodies are molecules that attack and destroy foreign organisms. For each type of organism, the immune system produces a special kind of antibody.

In a patient who has lupus, the immune system functions incorrectly. It thinks that the body's own cells are foreign organisms and releases antibodies to attack these cells the way it would attack bacteria and viruses. This causes tissues to become inflamed (red and swollen). They may even be killed by the attacking antibodies.

Lupus occurs in both males and females of all ages, but it is much more common in women. About 90 percent of all lupus cases occur in women. The majority of these women are of childbearing age. African Americans are more likely to develop lupus than Caucasians (whites).

CAUSES

The cause of lupus is unknown. Some researchers think that heredity may be a factor. Environmental factors also may be involved. In some cases, the symptoms of lupus become worse after exposure to sunlight, alfalfa sprouts, and certain medications. On rare occasions, a form of lupus can be caused by medications. Some drugs used to treat heart problems are among these medications. The lupus usually disappears when the person stops taking the drug.

Lupus: Words to Know

Autoimmune disorder:

A condition in which a person's immune system mistakes the body's own tissues for foreign invaders and begins to make antibodies against them.

Immune system:

A network of cells and tissues that work together to protect the body against foreign invaders, such as bacteria and viruses.

Psychosis:

Extremely disordered thinking accompanied by a poor sense of reality.

SYMPTOMS

The symptoms of lupus vary in seriousness. Sometimes they are quite mild, and sometimes they are quite severe. Typical symptoms include fever, fatigue, muscle pain, decreased appetite, and weight loss. The spleen and lymph nodes are often swollen. Other areas that may be affected by lupus include:

• Joints. Joint pain and disorders, such as arthritis (see arthritis entry), are common. About 90 percent of all lupus patients have such problems.
• Skin. Lupus may cause skin rashes on any part of the body. They usually occur on the face, scalp, chest, ears, back, arms, and legs. When they occur in the mouth, they form ulcers (open sores). Hair loss is common.
• Lungs. Lupus may cause inflammation of the pleura, the tissue that lines the lungs. The patient may experience coughing and shortness of breath.
• Heart and circulatory system. Lupus may cause inflammation of the tissue surrounding the heart (pericarditis; pronounced per-i-kar-DIE-tiss) or of the heart itself (myocarditis; pronounced my-o-kar-DIE-tiss). When this happens, various heart problems may develop, such as an irregular heartbeat (arrhythmia; pronounced uh-RITH-mee-uh), heart failure, and even sudden death. Blood clots often form in the blood vessels. These blood clots can break loose and cause a stroke (see stroke entry) or other complications.
• Nervous system. Headaches, seizures, personality changes, and psychosis (confused thinking) may occur.
• Kidneys. During a lupus attack, the body's kidney cells may begin to die. When this happens, the kidney can no longer filter blood. Toxins (poisons) may build up in the kidney, causing it to stop functioning.
• Gastrointestinal (digestive) system. Patients may experience nausea, vomiting, diarrhea, and abdominal (stomach) pain. The lining of the stomach may also become inflamed.
• Eyes. The eyes may become red, sore, and dry. Inflammation of nerves in the eye may cause vision problems and blindness.

DIAGNOSIS

Lupus is usually difficult to diagnose, especially since many of its symptoms are similar to other diseases, such as rheumatoid arthritis (see arthritis entry) and multiple sclerosis (see multiple sclerosis entry). There is no one test that can be used to diagnose the disease. Blood tests can be used to look for certain kinds of antibodies. The most dependable of these tests may be correct 70 percent to 80 percent of the time.

Many doctors rely on a standard created by the American Rheumatism Association to diagnose lupus. According to this standard, a patient has to have four of eleven symptoms to be diagnosed with lupus. Those symptoms are:

• Butterfly rash, a distinctive type of facial rash
• Discoid rash, another distinctive type of facial rash
• Unusual sensitivity to light
• Ulcers in the mouth
• Arthritis
• Inflammation of the lining of the lungs or the lining around the heart
• Kidney damage
• Seizures or psychosis
• Low numbers of red blood cells or certain types of white blood cells
• The presence of certain kinds of immune cells
• The presence of certain kinds of antibodies

TREATMENT

Treatment of lupus depends on how serious a patient's case is. Mild cases may involve rashes and moderate pain. These cases can be treated with nonsteroidal anti-inflammatory drugs, such as aspirin and ibuprofen (pronounced i-byoo-PRO-fuhn, trade names Motrin, Advil). More serious rashes and joint problems may be treated with drugs also used to treat malaria (see malaria entry).

More serious symptoms may have to be treated with steroids. Steroids can reduce inflammation and swelling. They have some serious side effects, however, so they must be used with caution.

The most seriously ill patients may be treated with immunosuppressant drugs. Immunosuppressant drugs cause the immune system to shut down partially or completely. These drugs also have very serious side effects. With a weakened immune system, a patient is at risk for many other kinds of infections.

Other lupus treatments are designed for specific systems affected by the disease. For example, substances that thin the blood can be used if blood clots have formed. A person whose kidneys have begun to fail may require kidney dialysis or even a kidney transplantation. Kidney dialysis is a process in which a machine artificially cleanses a person's blood.

Alternative Treatment

A number of alternative treatments have been suggested to help reduce the symptoms of lupus. These include acupuncture and massage for relieving the pain of sore joints and muscles. Patients can be taught to relax with techniques such as meditation and yoga. Hydrotherapy (water therapy) may also promote relaxation.

Proper nutrition may be an important factor in treating the symptoms of lupus. Some cases of the disease may be triggered by certain types of foods. Wheat, dairy products, and soy are the most common causes of the disease. Avoiding these foods may reduce the symptoms of lupus.

Some practitioners recommend nutritional supplements, such as vitamins B, C, and E, and minerals, such as magnesium, selenium, and zinc. They think these supplements may improve the general health of patients with lupus. Vitamin A can also be used to treat facial rashes.

Herbalists believe that certain herbs help relieve specific symptoms of lupus. They think that herbs can also help people develop a healthier outlook on life.

PROGNOSIS

The prognosis for lupus depends primarily on two factors: the systems affected and the degree of inflammation. Some patients experience mild symptoms or no symptoms at all over long periods of time. About 90 percent to 95 percent of all patients with lupus are still alive two years after diagnosis. Up to 75 percent of all patients survive twenty years or more.

The most common causes of death in the early years of lupus are infectious diseases and kidney failure. For people who have had the condition longer, the most common cause of death is blood clots.

PREVENTION

There is no way to avoid developing lupus. However, patients with the condition can often avoid the worst symptoms of the disease by maintaining a healthy diet, getting plenty of rest, avoiding stress, exercising regularly, and decreasing exposure to the sun. Patients can try to find out what factors seem to worsen their symptoms and then avoid those factors as much as possible.

FOR MORE INFORMATION

Books

Aaseng, Nathan. Autoimmune Diseases. New York: Franklin Watts, 1995.

Lahita, Robert G., and Robert H. Phillips. Lupus: Everything You Need to Know. Garden City Park, NY: Avery Publishing Group, 1998.

Wallace, Daniel J. The Lupus Book. New York: Oxford University Press, 1995.

Periodicals

Mann, Judy. "The Harsh Realities of Lupus." Washington Post (October 8, 1997): p. C12.

Umansky, Diane. "Living with Lupus." American Health for Women (June 1997): p. 92+.

Organizations

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

Lupus Foundation of America, Inc. 1300 Piccard Drive, Suite 200, Rockville, MD 20850–4303. (800) 558-0121. http://www.lupus.org.

Lupus Network. 230 Ranch Drive, Bridgeport, CT 06606. (203) 372-5795.

lupus (loo-pŭs) n. any of several chronic skin diseases. l. erythematosus (LE) an inflammatory autoimmune disease of connective tissue. In systemic LE (SLE) the skin and various internal organs are affected. Typically, there is a red scaly rash on the nose and cheeks; arthritis; and progressive damage to the kidneys. Often the heart, lungs, and brain are also affected. In a milder form, known as discoid LE (DLE), only the skin is affected. LE is treated with corticosteroids or immunosuppressant drugs. l. verrucosus a rare tuberculous infection of the skin – commonly the arm or hand – typified by warty lesions. It occurs in those who have been reinfected with tuberculosis. l. vulgaris a tuberculous infection of the skin that often starts in childhood, with dark red patches on the nose or cheek. Unless treated with antituberculous drugs lupus vulgaris spreads, ulcerates, and causes extensive scarring.
www.uklupus.co.uk Details of SLE from The Lupus Site