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Author Topic: Understanding SLE  (Read 18908 times)
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« on: March 31, 2007, 11:20:19 am »

Lupus, also called systemic lupus erythematosus or SLE, is a chronic autoimmune disease that affects tissue and organs throughout the body (e.g., joints, kidneys, skin, heart). The most common symptoms of the disease are painful, swollen joints; fatigue; and fever. The severity of the disease varies from mild to severe, and the symptoms, which also vary from patient to patient, often come and go over time.

The immune system is a complex network of organs, cells (e.g., leukocytes, lymphocytes, phagocytes, B cells, CD8+ cells), and proteins that protects the body from disease and illness. It attacks foreign organisms (e.g., germs), identifies and destroys abnormal cells, and flushes dead and damaged cells out of the body. In autoimmune diseases such as lupus, the immune system attacks normal cells in the body, causing damage and inflammation.

Incidence and Prevalence

Lupus is most common in women of childbearing age, but it can occur in men and women of any age. Approximately 90% of patients with systemic lupus erythematosus are women. SLE occurs about 3 times more often in African American women than Caucasian women and the condition is also more common in Hispanic, Asian, and Native American women.

Risk Factors and Causes

Lupus results from an abnormal immune system response, the cause of which is unknown. The condition appears to result from a combination of genetic (inherited), hormonal, and environmental factors. SLE may develop when the immune system mistakes normal cells in the body for foreign cells, possibly following infection with an invading organism (e.g., virus) that is similar to normal proteins in the body.

A number of genes related to SLE have been identified, but the inheritance rate is relatively low (ranges from 2–10%). Studies have shown that women are more likely to experience autoimmune disorders that result from genetic make-up than men, which may help to explain the higher prevalence of lupus in women.

The prevalence of lupus in women of childbearing age (i.e., after puberty and before menopause) may indicate that hormone production or the metabolism (physical and chemical processes) of hormones can also contribute to the condition.

It is unknown whether factors in the environment (e.g., chemicals, bacteria) contribute to the development of lupus. Exposure to sunlight appears to play a role in the exacerbation of symptoms (flares) associated with the disease.

In rare cases, medications (e.g., procainamide [Pronestyl®], hydralazine [Apresoline®]) can cause a syndrome that is similar to SLE (called drug-induced lupus). This condition, which has a genetic risk factor, causes general symptoms (e.g., fever, malaise), joint pain (arthralgia) and inflammation (polyarthritis), inflammation of the membranes surrounding the lungs (pleurisy) and heart (pericarditis).

Patients who experience drug-induced lupus are withdrawn from the medication and symptoms usually improve within a few weeks. In severe cases, corticosteroids are administered for 2 to 10 weeks.

Signs and Symptoms

Lupus follows many different courses and the symptoms vary from patient to patient. Symptoms may be mild or severe, and in many cases, the disease is marked by periods of remission (improvement in symptoms) and exacerbation, also called flares (worsening of symptoms).

Symptoms of lupus include the following:

    * Fatigue
    * Fever (usually less than 102° F)
    * General feeling of being unwell (malaise)
    * Hair loss
    * Muscle pain
    * Nausea and vomiting
    * Rash (commonly a "butterfly" rash across the cheeks and under the eyes)
    * Sensitivity to sunlight (called photosensitivity)
    * Sore or lesion (ulcer) in the mouth or nose, usually painless
    * Swelling and pain in the joints (e.g., hands, knees)
    * Swollen glands
    * Weight loss

Complications

Lupus, as well as medications used to treat the disease, may cause a number of complications. These complications, some of which can be severe, include the following:

    * Kidney damage (kidney failure is a leading cause of death in lupus patients)
    * Nervous system damage (e.g., headaches, dizziness, memory loss, seizures)
    * Cardiovascular damage (e.g., inflammation of the heart [pericarditis, endocarditis, myocarditis] and blood vessels; increased risk for heart attack)
    * Lung damage (e.g., pleurisy; increased risk for pneumonia)
    * Damage to bones (avascular necrosis caused by diminished blood supply to the bones)
    * Increased risk for cancer (e.g., non-Hodgkin's lymphoma, lung cancer, liver cancer)
    * Increased risk for infection (caused by the disease as well as the medications used to treat the disease)

During pregnancy, women with SLE have an increased risk for miscarriage and complications, such as diabetes and high blood pressure. Women with lupus also may not tolerate some types of birth control (e.g., birth control pills, intrauterine devices [IUDs]) well.

Diagnosis

The American College of Rheumatology has established the criteria for the classification of lupus. SLE is diagnosed if more than 4 of the following are present.

    * Malar rash (butterfly-shaped rash across the cheeks and nose)
    * Rash (raised red patches on the skin)
    * Photosensitivity (rash caused by exposure to sunlight)
    * Mouth or nose ulcers (usually painless)
    * Non-erosive arthritis (does not destroy the bones surrounding the joints), which occurs in 2 or more joints and causes tenderness, swelling, or effusion (increased amounts of fluid)
    * Cardiopulmonary involvement (e.g., inflammation of the lining around the heart [pericarditis] and/or the lungs [pleurisy])
    * Neurological disorder (e.g., seizures, psychosis, cognitive dysfunction)
    * Kidney (renal) disorder (e.g., excessive protein in the urine [proteinuria], cellular casts in the urine)
    * Blood disorder (e.g., hemolytic anemia, low white blood cell count, low platelet count)
    * Immunologic disorder (e.g., antibodies to double stranded DNA, antibodies to Sm, antibodies to cardiolipin)
    * Antinuclear antibodies (ANA; positive test in absence of drugs known to induce a positive test)

Laboratory tests

There is no test that is used specifically to diagnose SLE; however, the disease may affect a number of test results.

Most patients with lupus test positive for anti-nuclear antibody (ANA); however, as many as 20% of healthy women also test positive for this antibody, so the test is not definitive for lupus.

Depending on which organ systems are affected, additional laboratory tests can be used to detect a specific abnormality (e.g., an elevated red blood cell count in the urine if the kidneys are affected) caused by lupus.

Treatment

There is no cure for lupus, and the goals of treatment are to minimize symptoms, to reduce inflammation in the body, and to maintain function. Because the course of the disease varies from patient to patient, there are a number of different treatment plans. In many cases, preventative treatment can reduce the frequency and severity of flares (periods of worsening symptoms).

It is important for patients with lupus to avoid excessive sun exposure, wear sunscreen regularly, and avoid taking Echinacea (herb that stimulates the immune system).

It also is recommended that patients:

    * Avoid smoking
    * Have regular medical checkups
    * Limit alcohol intake
    * Take medication as prescribed

In most cases, women with lupus are unable to tolerate oral contraceptives (birth control pills) or estrogen replacement therapy (ERT). Sulfonamide, which is present in some antibiotics, also has been shown to cause flares in patients with SLE.

Medications

The types of medications used to treat lupus depend on which organs are affected and on the severity of symptoms. Medications that are commonly used include the following:

    * Non-steroidal anti-inflammatory drugs (NSAIDs; e.g., aspirin, ibuprofen [Advil®, Motrin®], naproxen [Naprosyn®], indomethacin [Indocin®])
    * Acetaminophen (e.g., Tylenol®)
    * Corticosteroids (e.g., Prednisone®)
    * Antimalarials (e.g., chloroquine [Aralen®], hydroxychloroquine [Plaquenil®])
    * Immunomodulating drugs (e.g., azathioprine [Imuran®], cyclophosphamide [Cytoxan®])

NSAIDs are used to treat muscle and joint pain and inflammation. These drugs may cause gastrointestinal side effects (e.g., stomach pain, gastrointestinal bleeding) and should be taken with meals. Antacids and other medications, such as Cytotec® and the newer drug Arthrotec® may further reduce the risk for these side effects.

Corticosteroids, which are hormones that reduce inflammation and suppress the immune system, cause a number of side effects (e.g., weight gain, high blood pressure, risk for infection). It is important to regulate the dose of these medicines to maximize the benefit and reduce the risk for adverse effects. The risks are higher when the medication is taken for a long period of time or at high doses.

Antimalarials may be prescribed for skin and joint symptoms of lupus. These medications rarely cause side effects, but they may affect the eyes. Patients who are taking antimalarials should have their eyes examined regularly (every 6 months to 1 year).

Immunomodulating drugs are immunosuppressive medications that reduce inflammation and suppress the immune system. Side effects include anemia (low red blood cell count), low white blood cell count, and an increased risk for infection. Patients using these drugs may also develop a predisposition to cancer in the future.

Intravenous immunoglobulin injections, which block production of specific antibodies, may be given to lupus patients on a regular basis to increase platelets (blood cells that aid in coagulation).

Other Treatments

If the kidneys are involved, a biopsy (surgical removal of a small piece of tissue for microscopic evaluation) is performed to determine what type of disease process is occurring. This information is necessary to develop the correct treatment plan.

If renal failure occurs, hemodialysis is the preferred treatment over peritoneal dialysis. Patients who experience kidney failure can be considered for renal transplant because the risk for development of the disease in the transplanted kidney is low.

Severe cases of inflammation in and around the lungs and heart may require surgical drainage, especially if fluid surrounding the heart decreases its ability to pump blood (called cardiac tamponade).

Prognosis (Outcome)

The outcome for patients with SLE has improved over recent years. According to the National Institutes of Health (NIH) the 10-year survival rate for patients with the condition is more than 85%. Patients with severe complications have a worse prognosis in terms of survival and disability. Women with SLE who become pregnant are often able to carry the pregnancy safely to term and deliver healthy infants, as long as severe kidney or heart disease is not present and the disease is being managed.

Source: http://www.rheumatologychannel.com/lupus/
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