Lupus is an autoimmune disease that causes chronic inflammation (swelling). Autoimmune conditions occur when the immune system attacks the body's own cells because they are mistaken for antigens (foreign substance that causes an immune response). Autoimmunity is present in all healthy individuals, to some extent. This is the result of genetically defective immune cells, which may develop during cellular reproduction. However, healthy individuals are able to suppress defective lymphocytes (immune system cells) that mistakenly destroy body cells.
Patients who have lupus can experience symptoms in any area of the body, including the skin, heart, lungs, kidneys, joints and/or nervous system. When only the skin is involved, the condition is called discoid lupus. When internal organs are involved, the condition is called systemic lupus erythematosus (SLE). The condition is not always progressive. Researchers estimate that about 10% of discoid lupus patients eventually develop SLE.
While the exact cause of lupus remains unknown, researchers believe that genetics, certain medications, hormones, viruses or ultraviolet light may contribute to the development of the disease.
Researchers estimate that women are eight times more likely to develop either discoid or systemic lupus than men, which suggest that hormones play a role in the pathogenesis of the disease. While the disease can occur at any age, it usually develops in individuals who are 20 to 45 years old. It is more frequent in African Americans and individuals of Japanese or Chinese descent, which suggests that genetics may be involved.
Researchers estimate that about one out of 10,000 Americans develops SLE each year.
There is currently no cure for lupus. Instead, treatment focuses on alleviating the symptoms of the disease. Patients who have mild symptoms may not need any treatment, or they may require intermittent use of anti-inflammatory medications (like ibuprofen). Individuals who have serious cases of lupus, which involve internal organ damage, may require high doses of corticosteroids, along with other medications that suppress the body's immune system (like methotrexate).
Prognosis varies depending on whether the disease affects internal organs. If the disease is limited to the skin, patients can live relatively normal lives with minimal problems. However, patients who have more severe lupus may experience kidney failure, heart attack or stroke.
The exact cause of lupus remains unknown. Researchers believe that genetics may be involved because individuals who have a family history of lupus are more likely to develop the disease than those who do not. Also, it is more common in African Americans and individuals of Japanese or Chinese descent.
The use of certain medications has also been associated with systemic lupus erythematosus (SLE). It is estimated that about 5% of SLE cases are drug-induced. About 90% of drug-induced lupus cases occur as side effects hydralazine, guanidine, procainamide, phenytoin, isoniazide or d-penicillamine. Drug-induced lupus resolves once the offending medication is discontinued.
Since women are eight times more likely to develop lupus than men, it has been suggested that hormones play a role in disease development. In addition, women often experience a worsening of symptoms before their menstrual periods. Ongoing research is being conducted to understand this relationship.
Some researchers also believe that the immune system in lupus patients is more easily stimulated by external factors like viruses or ultraviolet light. Many cases of lupus have been reported after exposure to such factors.
General: When only the skin is involved, the condition is called discoid lupus. When internal organs are involved, the condition is called systemic lupus erythematosus (SLE). One or more organs may be involved with SLE. The condition is not always progressive. Researchers estimate that about 10% of discoid lupus patients eventually develop SLE. When lupus develops, it may be difficult to diagnose because initial symptoms are very general and often include fatigue, fever and muscle and joint pain.
Musculoskeletal system: Some lupus patients may experience joint pains (especially of the hand, knees and wrists), muscle weakness and muscle pain.
Skin and hair: More than 90% of lupus patients experience symptoms that affect the skin. The classic lupus rash, which is characterized by reddened cheeks and nose (butterfly rash), is usually triggered by sun exposure. Wearing sunscreen and limiting sun exposure reduces the appearance of the rash. Discoid lupus causes red and scaly patches of skin on the face and scalp that can lead to scarring and temporary hair loss. Patients who are taking immunosuppressive medications may also experience hair loss as a side effect of the medication.
Kidneys: More than half of SLE patients experience kidney involvement. High blood pressure or blood in the urine may signal kidney disease.
Heart and blood vessels: Patients may develop pericarditis (inflammation of the sac that surrounds the heart), which may cause chest pain. Abnormal tissue growth can form on the heart valves. Hardening of the arteries can lead to chest pain and heart attacks. Some patients may not receive enough blood supply to their hands when they are exposed to cold temperatures. This condition, called Raynaud's phenomenon, causes whiteness and blueness in the fingers.
Nervous system: About 15% of SLE patients experience brain and nerve problems. Common symptoms include seizures, nerve paralysis, severe depression, psychosis (loss of contact with reality) and strokes. In rare cases, patients may experience spinal cord inflammation, which can lead to paralysis.
Lungs: More than half of SLE patients experience symptoms of lung involvement. The most common problem is pleurisy (inflammation of the lining of the lung), which can lead to chest pain and shortness of breath. Pleural effusions, which are collections of water between the lung and chest wall, may also occur.
Blood and lymph system: About half of lupus patients are anemic (low red blood cell counts), and up to half have thrombocytopenia (low platelet counts) and leukopenia (low white blood cell count). Common symptoms include bleeding, bruising and blood clots.
Gastrointestinal tract: Many SLE patients develop painless ulcers in the mouth and nose. When lupus involves the intestines, patients often experience abdominal pain.
General: The American College of Rheumatology has developed 11 criteria for the diagnosis of lupus. Individuals are diagnosed if they meet four of the 11 criteria.
Malar rash: A malar rash is a butterfly-shaped, red rash on the cheek and nose. The rash may be flat or raised.
Discoid rash: A discoid rash is characterized by red and scaly patches of skin on the face and scalp that can lead to scarring and temporary hair loss
Photosensitivity: Photosensitivity (sensitivity to light) occurs when the patient experiences a skin rash in response to ultraviolet light or sun exposure.
Oral or nasal ulcers: Painless ulcers may be observed in the mouth or nose.
Arthritis: Patients may experience swollen or tender joints.
Serositis: If serositis, or inflammation of the membranes that cover the lung, heart and abdomen, is observed, lupus may be indicated.
Kidney disease: High blood pressure, loss of protein in the urine or a microscopic analysis of the urine demonstrates inflammation of the kidneys. An ultra sound or computerized tomography (CT) scan may be conducted to measure the size the kidney and estimate blood flow to the kidneys.
Neurologic disorder: Neurologic disorders, which may cause seizures, nerve paralysis, severe depression, psychosis (loss of contact with reality) and strokes, may be observed in lupus patients.
Blood disorder: A complete blood count may be conducted to determine whether the patient has low blood counts. Lupus patients may have low red blood cell counts (anemia), low platelet counts (thrombocytopenia), or low white blood cell counts (leukopenia).
Immunologic disorder: Patients are tested to determine whether they have antibodies to DNA, a nuclear protein (Sm) or phospholipids.
Positive antinuclear antibody: The fluorescent antinuclear antibody test (FANA) is used to determine whether the patient has autoantibodies. Autoantibodies are defective immune cells that mistake body cells for foreign cells. To measure results, human tissue cells (HEp-2 cell line) are grown in culture and applied to a microscope slide. The slides are then treated with methyl alcohol to make the cells permeable before they are combined with the patient's blood. The cells are incubated with fluorescent antibodies that detect the binding of human antibodies to the cells. The slide is then viewed with a fluorescence microscope. Staining intensity and the pattern of binding are scored at various dilutions.
Up to up to 98% of people with lupus have a positive FANA test result. A positive test result may suggest an autoimmune disease, but further testing is needed to make a specific diagnosis. FANA test results can be positive in people who do not have autoimmune diseases. While a false positive result is uncommon, the frequency increases as people get older. A false negative result is also possible, but it is uncommon.
General: There is currently no cure for lupus. Instead, treatment focuses on alleviating the symptoms of the disease. Patients who have mild symptoms may not need any treatment, or they may require intermittent anti-inflammatory medications. Individuals who have serious cases of lupus, that involve internal organ damage, may require high doses of corticosteroids along with other medications that suppress the body's immune system. Drug-induced lupus resolves once the offending medication is discontinued.
Corticosteroids: Corticosteroids like methylprednisolone (Adlone©, Medrol©, Solu-Medrol© or Depopred©) and prednisone (Deltasone©, Orasone© or Meticorten©) have been used to suppress the body's immune system and decrease inflammation in patients who have severe lupus.
Immunosuppressants: Immunosuppressants like methotrexate (Folex PFS© or Rheumatrex©), cyclophosphamide (Cytoxan©), or azathioprine (Imuran©) have been used to suppress the body's immune system.
Nonsteroidal anti-inflammatory drugs (NSAIDs): Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen (Advil© or Motrin©) have been used to decrease mild to moderate inflammation and pain associated with lupus.
Antimalarial drugs: The mechanism of action of antimalarial agents in systemic lupus erythematosus (SLE) remains unknown. While they do not cause general immunosuppression, antimalarial agents like hydroxychloroquine (Plaquenil©) and chloroquine (Aralen© Hydrochloride or Aralen© Phosphate) have been shown to effectively treat lupus skin rashes and joint or muscle pain. In addition, hydroxychloroquine has been shown to decrease the frequency of flares in patients with SLE.
Sunscreen: Patients who are sensitive to sun exposure should wear ultraviolet-blocking sunscreens to prevent or reduce the development of a skin rash.
Good scientific evidence
: The majority of clinical trials investigating the effect of DHEA for systemic lupus erythematosus (SLE) support its use as an adjunct treatment. Additional research is needed to confirm these results.
Avoid if allergic to DHEA. Avoid with a history of seizures. Use cautiously with adrenal or thyroid disorders. Use cautiously if taking anticoagulants or drugs, herbs, or supplements that treat diabetes, heart disease, seizures, or stroke. Stop use two weeks before and immediately after surgery/dental/diagnostic procedures with bleeding risks. Avoid if pregnant or breastfeeding.
Unclear or conflicting scientific evidence
: Preliminary study suggests that copper offers no benefit to individuals with systemic lupus erythematosus (SLE). Further research is required.
Avoid if allergic or hypersensitive to copper. Avoid use of copper supplements during the early phase of recovery from diarrhea. Avoid with a high amount of copper in the blood (hypercupremia), genetic disorders affecting copper metabolism (e.g. Wilson's disease, Indian childhood cirrhosis, or idiopathic copper toxicosis), or HIV/AIDS. Use cautiously with water containing copper concentrations greater than 6 milligrams per liter. Use cautiously with anemia (low red blood cell count), arthralgias (painful joints), or myalgias (muscle pain). Use cautiously if taking oral contraceptives. Use cautiously if at risk for selenium deficiency. Pregnant and breastfeeding women should not consume doses that exceed the recommended dietary allowance (RDA).
Flaxseed and flaxseed oil
: Flaxseed and flaxseed oil/linseed oil are rich sources of the essential fatty acid alpha-linolenic acid (omega-6). Alpha-linolenic acid is a building block in the body for omega-3 fatty acids. Flaxseed oil should be refrigerated at all times. There is currently a lack of strong evidence available on the use of flaxseed for lupus nephritis. More research is needed before a firm recommendation can be made.
Flaxseed has been well-tolerated in studies for up to four months. Avoid if allergic to flaxseed, flaxseed oil, or other plants of the Linaceae family. Avoid with prostate cancer, breast cancer, uterine cancer, or endometriosis. Avoid ingestion of immature flaxseed pods. Avoid large amounts of flaxseed by mouth and mix with plenty of water or liquid. Avoid flaxseed with a history of esophageal stricture, ileus (loss of bowel motility), gastrointestinal stricture or bowel obstruction. Avoid with a history of acute or chronic diarrhea, irritable bowel syndrome, diverticulitis (bowel inflammation), or inflammatory bowel disease. Avoid topical flaxseed in open wounds or abraded skin surfaces. Use cautiously with a history of a bleeding disorders, high triglyceride levels, diabetes, mania, seizures, or asthma. Use cautiously if taking drugs that increase the risk of bleeding or nonsteroidal anti-inflammatory drugs (NSAIDs). Avoid if pregnant or breastfeeding.
Omega-3 fatty acids
: It has been suggested that omega-3 fatty acids may help alleviate symptoms of systemic lupus erythematosus SLE. However, scientific evidence is inconclusive. Further research is warranted before a firm conclusion can be made.
Avoid if allergic or hypersensitive to fish, omega-3 fatty acid products that come from fish, nuts, linolenic acid, or omega-3 fatty acid products that come from nuts. Avoid during active bleeding. Use cautiously with bleeding disorders, diabetes, low blood pressure, or if taking drugs, herbs or supplements that treat any such conditions. Use cautiously before surgery. Pregnant and breastfeeding women should not consume doses that exceed the recommended dietary allowance (RDA).
: There is conflicting evidence as to whether or not brief supportive-expressive group psychotherapy reduces psychological distress and medical symptoms and improves quality of life of women with systemic lupus erythematosus (SLE). Further studies are needed before a firm conclusion can be drawn.
Psychotherapy cannot always fix mental or emotional conditions. Psychiatric drugs are sometimes needed. In some cases, symptoms may worsen if the proper medication is not taken. Not all therapists are qualified to work with all problems. Use cautiously with serious mental illness or some medical conditions because some forms of psychotherapy may stir up strong emotional feelings and expression.
: Pycnogenol© is the patented trade name for a water extract of the bark of the French maritime pine (Pinus pinaster ssp. atlantica). Pycnogenol© may be useful as a second-line therapy to reduce inflammation associated with systemic lupus erythematosus (SLE). Further research is needed before a firm conclusion can be made.
Avoid if allergic or hypersensitive to pycnogenol, its components, or members of the Pinaceae family. Use cautiously with diabetes, hypoglycemia (low blood sugar), or bleeding disorders. Use cautiously if taking hypolipidemics (cholesterol-lowering medications), medications that may increase the risk of bleeding, hypertensive (high blood pressure) medications, or immune stimulating or inhibiting drugs. Avoid if pregnant or breastfeeding.
: Preliminary results indicate that articular and cutaneous symptoms associated with systemic lupus erythematosus may be improved with thymus extract use. Well designed clinical trials are required before thymus extract can be recommended for this use.
Avoid if allergic or hypersensitive to thymus extracts. Use bovine thymus extract supplements cautiously due to potential for exposure to the virus that causes "mad cow disease." Avoid use with an organ transplant or other forms of allografts or xenografts. Avoid if receiving immunosuppressive therapy, with thymic tumors, myasthenia gravis (neuromuscular disorder), untreated hypothyroidism, or if taking hormonal therapy. Avoid if pregnant or breastfeeding; thymic extract increases human sperm motility and progression.
Since the exact cause of lupus remains unknown, there is currently no known method of prevention.
This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com).
- American College of Rheumatology. Systemic Lupus Erythematosus. www.rheumatology.org. Accessed April 7, 2009.
- Arthritis Foundation. Lupus/Systemic Lupus Erythematosus (SLE). www.arthritis.org. Accessed April 7, 2009.
- Lupus Foundation of America. www.lupus.org. Accessed April 7, 2009.
- National Institute of Arthritis and Musculoskeletal and Skin Diseases. www.niams.nih.gov. Accessed April 7, 2009.
- Natural Standard: The Authority on Integrative Medicine. www.naturalstandard.com. Copyright © 2009. Accessed April 7, 2009.
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