Lupus

Introduction

Individuals with lupus are in a very real sense allergic to themselves. Lupus causes the body of an affected individual to attack its own cells and tissues, causing pain, inflammation and even organ damage to the sufferer. Read on to learn more about the four forms of lupus including causes and treatment.

What is it?

Lupus is a chronic inflammatory disease that can affect many organs, including the joints, skin, kidneys, heart, lungs, blood vessels, and brain. Depending on the type of lupus and the organs that are affected, the disease can be mild - as it is for most people - or very severe or even life threatening. The more body systems involved, the worse the prognosis. Diagnosis is sometimes difficult because the disease appears differently in each individual.

Lupus is classified as an autoimmune disease. Individuals with lupus have an impaired immune system that causes their bodies to produce antibodies called autoantibodies that inappropriately attack their own healthy tissues and cells. The autoantibodies contribute to inflammation and organ damage. Why the body loses its ability to differentiate between its own normal tissue cells and foreign cells is not well understood.

You can learn about the various types of lupus by clicking on one or more of the links below:

Systemic lupus erythematous (SLE)

Systemic lupus erythematous (SLE) is the predominant form of lupus affecting 70% of all people who have the disease. SLE is so named because it can affect almost any organ system of the body. For this reason, SLE is usually more severe than cutaneous lupus. The symptoms of SLE often vary from person to person. For some individuals, only the skin and joints are involved. For others, the joints, lungs, kidneys, blood, or other organs or tissues may be affected. Women have a greater risk of developing SLE than men by approximately 10 to 1.

Cutaneous lupus

Cutaneous lupus accounts for about 10% of all lupus cases. It affects only the skin and can create various kinds of skin lesions. A red, raised, scaly, coin-shaped rash is the classic rash that appears with this form of lupus. This localized rash occurs most frequently on the face, although it may appear on other parts of the body as well. Scarring often develops as the rash heals. Because exposure to ultraviolet light often triggers cutaneous lupus, it is recommended that individuals with this form limit their sun exposure and use sunscreen when out of doors. Some people with cutaneous lupus go on to develop systemic lupus erythematous (SLE).

Drug-induced lupus

Drug-induced lupus is a rare form of lupus that is reversible. It can affect individuals who take certain medications (for example, procainamide, hydralazine, isoniazid, quinidine, and phenytoin). Usually individuals have taken these medications for at least three to six months before develops symptoms that mimic SLE. These symptoms disappear within weeks to months after the affected individual discontinues the drug. Because the medications that can trigger drug-induced lupus are prescribed more frequently for men than for women, this type of lupus is more prevalent among men.

Neonatal lupus

Neonatal lupus is a rare form of lupus that appears a few days to weeks after birth in infants of mothers who have lupus. It typically disappears spontaneously, leaving no scarring or other aftereffects. The most common symptom is a rash that appears following exposure to sunlight, typically scattered over the body. A biopsy is needed to confirm a diagnosis of neonatal lupus, because the rash looks like many other rashes that newborns can experience. In rare situations, serious heart defects and liver abnormalities are associated with neonatal lupus. Infants with neonatal lupus typically do not go on to develop systemic lupus erythematous (SLE).

What causes it?

The cause of lupus is not known, although researchers are investigating several theories. It is thought that a combination of genetic, environmental, and possibly hormonal factors may contribute to the development of the disease. Some believe that the cause may differ from one individual to another.

Other factors - such as various medications, chemicals, and infections (both bacterial and viral), as well as emotional stress - may trigger a response that causes the immune system to malfunction. The fact that lupus can run in families indicates that it has a genetic basis. Studies have shown that lupus is more likely to affect identical twins, which share the exact same set of genes, than two non-identical siblings. Research is being conducted to determine if an individual's risk of developing lupus is greater if more than one family member suffers from the disease.

Who has it?

Approximately 1.7 million cases of lupus are reported per year in the United States alone. Lupus can strike men and women of all ages. However, for 80% of individuals with SLE - the most common form of lupus - the onset of the disease occurs between 15 and 45 years of age. Nearly 90% of individuals diagnosed with SLE are women, and black women are three times as likely to develop lupus as are white women. Women of Hispanic, Asian, and Native American descent are also at greater risk for lupus than white women.

No single test is available to make a definitive diagnosis of lupus. Several tests are usually conducted when lupus is suspected. The most common screening test, the antinuclear antibody (ANA) test, is typically performed when an autoimmune or rheumatic disease, particularly lupus, is suspected. The ANA test can help determine whether autoantibodies, which react against the patient's own tissues and cells, are present.

If an ANA test result is positive, additional testing is performed to confirm a diagnosis of lupus. For example, a biopsy may be performed to test the damaged organs. Other laboratory tests used both in the diagnosis of lupus and to monitor its progression include a complete blood count, urinalysis, blood chemistries, and erythrocyte sedimentation rate (ESR).

What are the risk factors?

Risk factors are characteristics that predispose people to develop a condition or illness. Common risk factors for lupus include:

• Being a woman: SLE occurs eight to 10 times more frequently among women than among men - perhaps because of hormonal differences in men and women. No scientific study has confirmed that female hormones are a root cause, but medical professionals have reported lupus flare-ups in women who are experiencing hormonal fluctuations (as with pregnancy and the menstrual cycle, for example) or who use oral contraceptives or hormone replacement therapies.

• Being an African American, Hispanic, Asian, or Native American: SLE is three times more common among black women than white women. Women of Hispanic, Asian, and Native American descent also are more likely to get SLE than white women.

• Family history: Because lupus has a genetic base, the risk for developing lupus may increase if a first-degree relative, such as a mother or a father, has lupus.

What are the symptoms?

Lupus is a disease that tends to cycle from flares (worsening symptoms that signal increased disease activity) to remission (periods during which symptoms are minimal or totally absent). Flares can be associated with the development of new symptoms or the onset of another form of lupus. With treatment, the disease can go into remission.

Although it is not possible to predict how long a flare will last, warning signs include:

• Increased fatigue
• High fever
• Increased pain in the joints and muscle
• Development or worsening of a rash
• Upset stomach
• Headache or dizziness

The symptoms of lupus often vary depending on the individual and the form of lupus present. They include rashes, Raynaud's phenomenon, flu-like symptoms, swollen or painful joints, pulmonary pain or inflammation, cardiac problems, gastrointestinal symptoms, and psychiatric symptoms.

• Rashes: Individuals with cutaneous lupus typically have a coin-shaped rash. A "butterfly rash," the most common manifestation of SLE, is generally observed after exposure to the sun. A butterfly rash is a distinctive, butterfly-patterned rash on the face. The wings appear beneath both eyes, and the body of the butterfly covers the bridge of the nose. Because individuals with lupus are often sensitive to sunlight, proper precautions should be taken to prevent sunburn. To learn more about specific measures for preventing sunburn, see the "How is it Treated" section, and then click on the "Helping Yourself" link.

• Raynaud's phenomenon: Raynaud's phenomenon is another symptom that individuals with SLE may experience. Decreased blood flow to the fingers results in a change in finger color (for example, redness, bluish or purplish discoloration, loss of color), mainly on exposure to cold or with emotional stress.

• Flu-like symptoms: Individuals with SLE commonly experience weight loss, loss of appetite, abdominal pain, extreme fatigue, and fever.

• Swollen or painful joints: Swollen joints and sharp pain in the joints are frequently reported by individuals who have recently begun experiencing lupus-related symptoms.

• Pulmonary pain and inflammation: Individuals with lupus may experience chest pain on breathing, which may be associated with underlying lung involvement. Some people experience inflammation of the lungs, or lupus pneumonitis, which is often characterized by fever, productive cough, and painful and rapid breathing.

• Cardiac problems: Cardiac manifestations of lupus are relatively uncommon. When present, they include infection of the heart muscle, heart murmur, and irregular heart rhythms. Symptoms include chest pain, shortness of breath, fatigue, and leg swelling.

• Gastrointestinal symptoms: Symptoms associated with gastrointestinal manifestations, which are relatively uncommon, include nausea, abdominal pain, difficulty swallowing, and bloody stools. Very infrequently, an enlarged liver or spleen and pancreatitis (inflammation of the pancreas) may be present in individuals with SLE.

• Psychiatric symptoms: Psychiatric symptoms - such as psychosis, depression, seizures, headache, dizziness, memory disturbances, and strokes - are common manifestations of SLE and affect individuals differently.

The following table shows the occurrence of typical symptoms among individuals with lupus.

Lupus: Symptoms and Occurrence among Affected Individuals

Source: Lupus Foundation of America. Available at: http://www.lupus.org.

How is it treated?

There is no cure for lupus, but individuals with the disease can find the best treatment and usually maintain their quality of life by working closely with their health care providers. Desired treatment goals include managing symptoms, inducing remission during times of disease flare, and maintaining remission for as long as possible.

Several types of drugs can be used to achieve treatment goals, but treatment must be individualized according to each person's particular symptoms. Finding the best treatment regimen tends to be a trial-and-error process because individuals respond differently to medications. It is therefore quite common for physicians to recommend one medication, monitor the relief it provides the patient and, if the desired result is not achieved, to change the dosage or prescribe another medication. Because some treatments may cause harmful side effects, it is important that individuals undergoing treatment for lupus promptly report new symptoms or side effects to their health care providers.

Drug therapy usually is targeted at reducing inflammation, the cause of most lupus symptoms. Drugs used to treat the disease include nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, disease modifying antirheumatic drugs (DMARDs), and cytotoxic drugs.

• NSAIDs: NSAIDs (either over-the-counter or prescription strength) are often recommended to alleviate fever, muscle pain, and joint pain and swelling. Individuals with a mild form of lupus may find an NSAID the only medication they need to relieve their symptoms, whereas individuals with more active or severe disease may require additional medications.

• Corticosteroids: Corticosteroids are the most common drugs used in lupus treatment because they reduce inflammation at a rapid speed and have been associated with a slowdown in the progression of the disease. The goal of corticosteroid therapy is to induce and maintain lupus remission using the lowest possible dose to avoid side effects, which can include fluid retention, muscle weakness, weight gain, increased blood sugar, and bone growth suppression (osteoporosis). Major organ involvement, however, may require higher doses of corticosteroids.

• DMARDs: DMARDs are frequently used in the treatment of lupus. They work by interfering with the immune system's function and are thought to protect against the damaging effects of ultraviolet light. DMARDs are effective in treating joint pain, skin rashes, fatigue, and inflammation of the lungs associated with lupus. Continuous use of DMARDs may prevent flares from recurring. In some cases, the use of DMARDs allows an individual to take lower doses of corticosteroids, decreasing the risk of side effects associated with high-dose corticosteroids.

• Cytotoxic drugs: Cytotoxic (cyto = cell; toxic = damage) drugs are usually prescribed for severe cases of lupus, when more than one major organ (kidneys, liver, brain, heart, lungs) is affected. These drugs work by targeting rapidly growing cells and therefore are useful in suppressing the cells involved in the hyperactive immune response.

Most individuals who have lupus are treated with NSAIDs, corticosteroids, and DMARDs, either alone or in combination.

To learn more about the drug classes used to treat lupus, click on the following links:

Helping Yourself

Diet: Individuals with lupus should eat a healthy diet. No specific lupus diet has been formulated. It is generally recommended that individuals with lupus consider following the American Heart Association or American Cancer Society diet. Alfalfa sprouts, a member of the legume family, contain an amino acid called L-canavanine. This amino acid can stimulate the immune system in people with lupus and increase inflammation. Therefore, patients with lupus should avoid eating alfalfa sprouts. Other legumes have a much lower concentration of L-canavanine and are safe to eat.

Exercise: Individuals with lupus are encouraged to exercise, particularly when lupus symptoms are inactive. Moderate activities such as walking, swimming, and bicycling can alleviate joint pain. Lupus patients should talk to their health care providers before beginning an exercise program.

Sleep: Individuals with lupus often suffer from fatigue during flare-ups. Especially during such times, it is important to get plenty of sleep. Schedule modifications may be required to permit the rest that sufferers need to regain their strength.

Medications: Individuals with lupus should take all medications prescribed by the treating physician and report to the physician or a pharmacist if any side effects are experienced or the medications do not control lupus symptoms.

Work schedule: Many individuals with lupus are able to work full-time, some can work only part-time, and still others may need to take a leave of absence during flare-ups.

Avoid smoking: Recent research suggests that smoking may be associated with an increased risk for developing lupus and increased disease activity in patients who already have SLE. Smoking also increases the risk of cardiovascular complications in patients with lupus.

Other Tips:

• Because ultraviolet light can trigger rashes, limit sun exposure and use sunscreen (SPF 15+). Avoid prolonged periods of exposure to sunlight, especially between 10 a.m. and 3 p.m., when the sun's rays are the most intense.

• Report warning signals to your health care provider as soon as possible.

• Limit stress and develop a support system of friends and family members.

• Avoid elective surgery until lupus is controlled or in remission.

• If your treatment includes corticosteroids or antimalarials, be sure to have the recommended annual eye exams.

What is on the horizon?

Although there is not a cure for lupus today, scientists are developing new and better ways to study lupus, with the dual goals of improving the quality of life for lupus sufferers and ultimately conquering the disease. Researchers are currently focusing on finding new treatment options for lupus, including treatments that minimize the use of corticosteroids, as well as more effective drug combinations. Researchers are also trying to gain a better understanding of the role genetics plays in lupus, which will provide insights that help in both the diagnosis and prevention of lupus.

Under study for women with SLE is dehydroepiandrosterone (DHEA) or Prestara, an investigational new drug. DHEA is the most abundant sex hormone in both men and women and has found to be deficient in patients with lupus. Supplementation with Prestara has shown to reduce flare-ups and steroid requirements. DHEA is also being studied to determine if it can be used to help prevent bone loss.

Investigational monoclonal antibodies, such as anti-C5 and anti-BLyS, target and inactivate specific processes of the immune system in order to prevent lupus flare-ups. In addition, the cancer medication rituximab, an anti-CD20 monoclonal antibody, has shown promise as an alternative therapy for lupus. In a small study, treatment with rituximab was associated with a reduction in lupus symptoms and was safe. However, more studies are needed to better determine its role in the treatment of lupus.

Early research involving stem cell transplantation (SCT) in patients with severe lupus has also shown promising results. A recent study showed that certain patients with lupus that did not respond to treatment with standard therapies had substantial improvement in disease activity several years following the transplant procedure, which used the patient's own stem cells. Additional studies are needed to compare the safety and effectiveness of SCT with standard treatments.

References

Arthritis Foundation home page. Available at: http://www.arthritis.org/conditions/DiseaseCenter/lupus.asp. Accessed August 15, 2007 and October 11, 2008.

Chang DM, Lan JL, Lin HY, Luo SF et al. Dehydroepiandrosterone treatment of women with mild-to-moderate systemic lupus erythematosus: a multicenter randomized, double-blind, placebo-controlled trial. Arthritis Rheum. 2002 Nov;46(11):2924-7.

Current treatment of cutaneous lupus erythematous. Dermatology (online journal). 2001;7(1):2. Available at: http://dermatology.cdlib.org/DOJvol7num1/transactions/lupus/werth.html. Accessed May 29, 2006 and October 11, 2008.

Delafuente JC and Cappuzzo KA. Systemic lupus erythematosus and other collagen vascular diseases. Pharmacotherapy: A Pathophysiologic Approach. 6th ed. DiPiro JT, Talbert RL, Yee GC, Matzke GR, Wells BG, Posey LM and others, eds. New York: McGraw-Hill; 2005.

Looney RJ, Anolik J, Sanz I. et al. B cells as therapeutic targets for rheumatic diseases. Curr Opin Rheumatol. 2004 May;16(3):180-5.

Looney RJ et al. B cell depletion as a novel treatment for systemic lupus erythematosus. Arthritis Rheum. 2004; 50(8): 2580-2589.

Lupus Foundation of America home page. Available at: http://www.lupus.org/. Accessed August 15, 2007 and October 11, 2008.

Lupus Site home page. Available at: http://www.uklupus.co.uk/. Accessed August 15, 2007 and October 11, 2008.

Lupus. The Mayo Clinic. Available at: http://www.mayoclinic.com/health/lupus/DS00115. Accessed August 15, 2007 and October 11, 2008.

Lupus. The Medline Plus. Available at: http://www.nlm.nih.gov/medlineplus/lupus.html. Accessed August 15, 2007 and October 11, 2008.

National Institute of Arthritis and Musculoskeletal and Skin Diseases. Available at: http://www.niams.nih.gov/hi/topics/lupus/slehandout/index.htm. Accessed August 15, 2007 and October 11, 2008.

Neonatal Lupus. Lupus Erythematous Federation home page. Available at: http://www.elef.rheumanet.org/newsletter/6/neonatal.htm. Accessed May 29, 2003.

Lupus Health Condition Last Updated: October 2008

Note: The above information is intended to supplement, not substitute for, the expertise and judgment of your physician, pharmacist, or other healthcare professional. It is not intended to diagnose a health condition, but it can be used as a guide to help you decide if you should seek professional treatment or to help you learn more about your condition once it has been diagnosed.

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