Definition and Types of Lupus
Lupus is a condition characterized by chronic inflammation of body tissues caused by autoimmune disease. Autoimmune diseases are diseases that occurs when the body's tissues are attacked by its own immune system. Immune system is a complex system in the body designed to combat / fight infectious agents, eg, bacteria, and other foreign invaders. One of the mechanisms used by the immune system to fight infections is the production of antibodies. Patients with lupus produce abnormal antibodies in their blood that target tissues within the body itself rather than foreign infectious agents. Because the antibodies and cells that accompany inflammation can involve tissues anywhere in the body, lupus has the potential to affect various areas of the body. Sometimes lupus can cause skin disease, heart, lungs, kidneys, joints, and / or nervous system. When only the skin is involved, the condition is called discoid lupus (discoid lupus). When internal organs are involved, the condition is called systemic lupus erythematosus (systemic lupus erythematosus, SLE).
Both discoid lupus and systemic lupus are more common in women than in men (approximately eight times more common). The disease can affect all ages but most commonly begins from age 20 to age 45. More frequently in oranr-African Americans and people of Chinese descent and Japanese.
Causes of Lupus
The precise reason for the abnormal autoimmunity that causes lupus is still unknown. Inherited genes, viruses, ultraviolet light, and drugs may all play the same role. Genetic factors increase the likelihood of developing autoimmune diseases, and autoimmune diseases such as lupus, rheumatoid arthritis, immune and thyroid disorders are more common among relatives of patients with lupus than the general population. Some scientists believe that the immune system in lupus is more easily stimulated by external factors like viruses or ultraviolet light. Sometimes, the symptoms of lupus can be precipitated or aggravated by only a brief period of sun exposure.
Also note that some women with SLE can experience worsening of their symptoms before their menstrual periods. These events, along with a female predominance of SLE, suggesting that female hormones play an important role in the expression of SLE. This hormonal relationship is an active area of studies by scientists who are running.
More recently, studies have demonstrated evidence that a key enzyme's failure to remove dead cells that may contribute to the development of SLE. DNase1 enzyme, normally eliminates the so-called "junk DNA" ("garbage DNA") and the debris of other cells by chopping them into tiny fragments for easier disposal. The researchers turned off the DNase1 gene in mice. The mice appeared healthy at birth but after six to eight months, the majority of mice without DNase1 showed signs of SLE. Thus, a genetic mutation in a gene that can disrupt the dump body cells may be involved in the onset of SLE.
Drug-induced lupus
Dozens of drugs have been reported to trigger SLE; however, more than 90% of lupus caused by the drug occurs as a side effect of one of the following six drugs: hydralazine (used for high blood pressure), quinidine and procainamide (used for arrhythmia / abnormal heart rhythm), phenytoin (used for epilepsy), isoniazid [(Nydrazid, Laniazid), used for tuberculosis / tuberculosis], d-penicillamine (used for rheumatoid arthritis). These drugs are known to stimulate the immune system and cause SLE. Fortunately, SLE is caused by the drug are rare (there are less than 5% of SLE among all patients with SLE) and usually disappear when the medication was discontinued.
Symptoms and Signs of Lupus
In discoid lupus, typically only the skin is involved. Reddened skin (skin rash) in discoid lupus is often found on the face and scalp. Generally, red and may have restrictions that stand out. Rash of discoid lupus is generally painless and not itchy, but the wounds that leave scars can cause permanent hair loss. With the passage of time, 5% -10% of patients with discoid lupus may develop SLE.
Patients with SLE may develop combinations of different symptoms and organ involvement. Common complaints include fatigue, mild fever, loss of appetite, sore muscles, arthritis, ulcers (ulcers) of the mouth and nose, face rash ("butterfly rash"), an unusual sensitivity to sunlight ( photosensitivity), inflammation of the lining surrounding the lungs (pleurisy) and heart (pericarditis), and poor circulation to the fingers and toes with exposure to cold (Raynaud's phenomenon).
More serious organ involvement with inflammation occurs in the brain, heart / liver, and kidneys. White blood cells and clotting factors can also be reduced in SLE, thereby increasing the risk of infection and bleeding.
More than half of patients with SLE develop a distinctive red rash face and flat on the bridge of his nose. Because of its shape, he is often referred to as the "butterfly rash" of SLE. Rash is not painful and does not itch. Rash advance along with inflammation in other organs can be precipitated or exacerbated by exposure to sunlight, a condition called photosensitivity. This photosensitivity can be accompanied by worsening of inflammation throughout the body, called a "flame (flare)" of the disease.
Most of the patients with SLE will develop arthritis during the course of their illness. Arthritis in SLE commonly involves swelling, pain, stiffness, and even deformation of the small joints of the hands, wrists, ankles, and legs. Sometimes, the arthritis of SLE can mimic that of rheumatoid arthritis (another autoimmune disease).
Inflammation of the muscles (myositis) can cause muscle pain and weakness. Inflammation of blood vessels (vasculitis) that supply oxygen to tissues, can cause isolated injury to a nerve, skin, or an internal organ. Blood vessels are composed of arteries that provide oxygen-rich blood to the tissues of the body and the veins that return blood that oxygen has been spent from the tissues to the lungs. Vasculitis is characterized by inflammation with damage to the walls of blood vessels. The damage is blocking the circulation of blood throughout the vessels and can cause injury to the tissues supplied by the vessels.
Inflammation of the lining of the lungs (pleurisy) and from the heart (pericarditis) can cause sharp chest pain. Chest pain is aggravated by cough, breathe deep, and changes in certain body positions. The heart muscle itself rarely can become inflamed (carditis). It has also been shown that young women with SLE have a significantly increased risk of heart attacks from coronary artery disease.
Renal inflammation in SLE can cause leakage of protein into the urine (urine), fluid retention, high blood pressure, and even kidney failure. With kidney failure, machines are needed to cleanse the blood of toxins that accumulate in a process called dialysis.
Involvement of the brain can lead to personal changes, thought disorders (psychosis), seizures and even coma. Damage to the nerves can cause numbness, tingling (tingling) and weakness of body parts involved or the feet and hands. Brain involvement is called cerebritis.
Many patients with SLE experience hair loss (alopecia). Often, this occurs simultaneously with an increase in disease activity.
Some patients with SLE have Raynaud's phenomenon. In these patients, the blood supply to the fingers and toes becomes interrupted upon exposure to cold air, causing a pale, bluish discoloration, and pain in fingers and toes are exposed.
Diagnosing Lupus
Because patients with SLE can have a great variety of symptoms and combinations of different organ involvement, no single test that establishes the diagnosis of systemic lupus. To help doctors improve the accuracy of diagnosis of SLE, eleven criteria established by the American Rheumatism Association. Eleven of these criteria are closely linked with the symptoms discussed above. Some patients who have SLE suspected may never develop enough criteria for a definitive diagnosis. Other patients accumulate enough criteria only after observation for months or years. When a person has four or more of these criteria, the diagnosis of SLE is highly recommended. Nevertheless, the diagnosis of SLE can be made in some manner in patients with only a few of these classic criteria.
11 criteria used to diagnose systemic lupus erythematosus (systemic lupus erythematosus) is:
* Malar (cheek on face) "butterfly" rash
* Discoid skin rash: patchy redness that can cause scarring (scarring)
* Photosensitivity: skin rash in reaction to sunlight exposure
* Ulcers of the mucous lining (mucus membrane ulcers): ulcers of the lining of the mouth, nose or throat
* Arthritis: two or more swelling and tender joints of the feet and hands
* Pleuritis / pericarditis: inflammation of the tissue lining around the heart or lungs, usually associated with chest pain with breathing
* Kidney abnormalities: abnormal amounts of urine protein or clumps of elements called cells casts
* Irritation of the brain (brain irritation): manifested by severe disturbances (seizures, convulsions) and / or psychosis
* Blood count abnormalities: low numbers of white blood cells or red blood, or platelets
* Immunologic disorder: tests abnormal immune antibodies, including anti-DNA or anti-Sm (Smith), a blood test false positive for syphilis, anticardiolipin antibodies, lupus anticoagulant, or positive LE prep test
* Antinuclear antibody: ANA antibody test positive
In addition to these 11 criteria, other tests can be helpful in evaluating patients with SLE to determine the severity of the organ involved. These include routine tests of blood to detect inflammation (for example, a test called the sedimentation rate), blood chemistry tests, a direct analysis of internal body fluids, and tissue biopsies. Abnormalities in body fluids and tissue samples (kidney, skin and nerve biopsies) can further support the diagnosis of SLE. Testing procedures are adequate for patients selected individually by the doctor.
Systemic Lupus Treatment
There is no permanent cure for SLE. The goal of treatment is to relieve symptoms and protect organs by decreasing inflammation and / or the level of autoimmune activity in the body. Many patients with mild symptoms may require no treatment or only intermittent administration of anti-inflammatory drugs. Those with more serious illness involving damage to internal organs may require high doses of corticosteroids in combination with other medications that suppress the body's immune system.
Patients with SLE need more rest during periods when the disease is active. Researchers reported that poor sleep quality is a significant factor in the development of fatigue in patients with SLE. These reports emphasize the importance for patients and doctors to refer to the quality of sleep and the effects of the depression behind it, lack of exercise, and strategies surrounding the self-care on overall health. During these periods, exercises are prescribed with caution is still essential to maintain muscle tone and range of motion of joints.
Nonsteroidal antiinflammatory drugs (NSAIDs) are helpful in reducing inflammation and pain in muscles, joints, and other tissues. Examples of NSAIDs include aspirin, ibuprofen (Motrin), naproxen (Naprosyn), and sulindac (Clinoril). Since individual response to NSAIDs varies among patients, it is common for a doctor to try different NSAIDs to find one that is most effective with the fewest side effects. Side effects The most common are stomach upset, abdominal pain, ulcers (ulcers), and even bleeding ulcers. NSAIDs are generally taken with food to reduce side effects. Sometimes, the drugs prevention ulcers when taking NSAIDs, such as misoprostol (Cytotec), are given simultaneously.
Corticosteroids are more powerful / effective than NSAIDs in reducing inflammation and restore function when active disease. Corticosteroids are particularly useful when the internal organs are involved. Corticosteroids can be administered orally, injected directly into the joints and other tissues, or inserted through the veins (intravenously). Unfortunately, corticosteroids have side effects serious if given in high doses for periods of time, and doctors will try to monitor the activity of the disease in order to use the lowest dose that is safe. Side effects of corticosteroids include weight gain, thinning of the bones and skin, infections, diabetes, facial swelling, cataracts, and death (necrosis) of large joints.
Hydroxychloroquine (Plaquenil) is an antimalarial drug has been found particularly effective for SLE patients with fatigue, skin and joint disease. Side effects include diarrhea, stomach disorders, and eye pigment changes. Eye pigment changes are rare, but require supervision (monitoring) by an eye doctor (ophthalmologist, eye specialist) during treatment with Plaquenil. Researchers have found that Plaquenil reduces significantly the frequency of abnormal blood clots in patients with systemic SLE. Moreover, the effect seemed independent of immune suppression, implying that Plaquenil can work immediately to prevent blood clot. This fascinating work highlights an important reason for patients and doctors to consider Plaquenil, especially for patients who are at some risk of blood clots in the veins and arteries, such as those with antibody- phospholipid antibodies (cardiolipin antibodies, lupus anticoagulant, and false positive VDRL). This means not only that Plaquenil reduces the chance to expand back of SLE, but it can also be advantageous in pengenceren blood to prevent excessive blood clotting are abnormal.
For resistant skin disease, drugs other antimalarials such as chloroquine (Aralen) or quinacrine, are considered and can be used in combination with hydroxychloroquine. Alternative medicines for skin diseases include dapsone and retinoic acid (Retin-A). Retin-A is often effective for such a form that is not common warts of the skin disease lupus. For more severe skin disease, drugs peneken immunity (immunosuppressive medications) are considered as below.
Drugs which signed the immunity (immunosuppressive medications) are also called cytotoxic drugs. Medications used to treat immune peneken patients with a manifestation-a manifestation of SLE with more severe damage to internal organs. Examples of immune peneken drugs including methotrexate (Rheumatrex, Trexall), azathioprine (Imuran), cyclophosphamide (Cytoxan), chlorambucil (Leukeran), and cyclosporine (Sandimmune). All drugs can suppress the immune peneken seriously blood cell counts and increased risk of infection and bleeding. Other side effects are typical for each drug. For example, Rheumatrex can cause liver toxicity, while Sandimmune can interfere with renal function.
In recent years, mycophenolate mofetil (Cellcept) has been used as an effective drug for lupus, especially when associated with kidney disease. Cellcept has been useful in reversing the active renal lupus (lupus renal disease) and in maintaining remission after that set. Low side effect profile that is more profitable than drugs that suppress the traditional immunity.
In patients with brain disease or serious kidney, plasmapheresis is sometimes used to remove antibodies and other immune substances from the blood to suppress immunity. Some SLE patients can develop a low platelet levels that seriously, thereby increasing the risk of excessive bleeding and spontaneous. Because the spleen (spleen) is believed to be the major site of platelet destruction, surgical removal of the spleen is sometimes performed to improve platelet levels. Other treatments include plasmapheresis and the use of male hormones. Plasmapheresis has also been used to remove proteins (cryoglobulins) that can lead to vasculitis. The final stage of SLE kidney damage requiring dialysis (dialysis) and / or a kidney transplant (kidney transplant).
Recent research indicates the benefits of rituximab (Rituxan) in treating lupus. Rituximab is an antibody infused intravenously to suppress a certain white blood cells, B cells, by reducing the amount in circulation. B cells have been found to play a central role in lupus activity, and when they are pressed, the disease tends toward remission.
At the meeting of the National Rheumatology in 2007, there is a paper presented suggest that the extra food from omega-3 fish oils in low doses can help patients by reducing lupus disease activity and possibility of reducing the risk of disease jantungk.
Prevent Lupus Activity
SLE without doubt is a potentially serious disease with involvement of many organ systems. However, it is very important to recognize that most patients with SLE run a full life, active, and healthy. Periodic increases in disease activity (flares) can generally be managed by a variety of drugs. Because ultraviolet light can accelerate and exacerbate flares, patients with systemic lupus should avoid sun exposure. Sun protective clothing and cover arms and legs can help. Dismissal of drugs with a sudden, especially corticosteroids, can also cause flares and should be avoided. Patients with SLE are at risk of infections increases, especially if they are taking corticosteroids or immunosuppressant drugs. Therefore, any fever that is not expected to be reported and evaluated.
The key to success is managing SLE regular contact and communication, allowing monitoring of symptoms, activities of disease, and treatment side effects.
Lupus affects Pregnancy
Patients with SLE who become pregnant are considered "high risk". Women with SLE who become pregnant require close observation during pregnancy and birth time. These include the fetal monitor the baby by obstetricians during pregnancy. These women may have an increased risk of miscarriage and can have flares of SLE during pregnancy. The presence of phospholipid antibodies, such as cardiolipin antibodies or lupus anticoagulant, in the blood can identify those patients who are at risk of miscarriage. Cardiolipin antibodies are associated with a tendency toward blood clotting. Patients with SLE who have cardiolipin antibodies or lupus anticoagulant may require blood-thinning drug-Oba (aspirin with or without heparin) during pregnancy to prevent miscarriage. Other treatments reported include the use of gamma globulin intravenously to selected patients with a history of miscarriages, premature and that the blood coagulant elements (platelets) are low during pregnancy. Pregnant women who have a blood clotting events previously possible benefit with continuation of blood thinners during and after pregnancy for a period of 6 to 12 weeks, at which time the risk of clotting associated with pregnancy seems to decrease. It has now been found that Plaquenil is safe for use during pregnancy treating SLE.
Future Lupus
Overall, forecasts for patients with systemic lupus become better every decade with the development of tests and treatments monitors are more accurate.
The role of the immune system in causing diseases become better understood through research. This knowledge will be applied to plan treatment methods are safer and more effective. For example, revise cecara complete immune system of patients premises treatments that are very aggressive while virtually removing immune system, is being evaluated. The studies now involve immune eradication with or without replacement of cells that could reshape the immune system (stem cell transplantation).
It should be noted that patients with SLE are at a somewhat increased risk of developing cancer. Cancer risk is most dramatic for blood cancers, like leukemia and lymphoma, but also increased for breast cancer. This risk may be related in part to changes in the immune system that are characteristic of SLE.
Women with SLE seems to be at increased risk of heart disease (CHD) according to recent reports. Women with SLE should be evaluated to minimize the risk factors of heart disease, such as increased blood cholesterol, quitting smoking, high blood pressure, and obesity.
DHEA (dehydroepiandrosterone) helps in reducing fatigue, improving thinking difficulties, and improve quality of life in patients with SLE. Recent research indicates that DHEA has been shown to improve or stabilize the signs and symptoms of SLE. DHEA is commonly available in health food stores, too, pharmacies, and in many food stores.
Prominent research has clearly shown that the drugs oral contraceptive does not increase the rate of flares of systemic lupus erythematosus (systemic lupus erythematosus). This important finding was in contrast with what it has been predicted for years. Now we can reassure women with lupus that if they take birth control pills, they do not increase their risk for the onset of lupus (SLE flares). Note: birth control pills or estrogen medications whatever remains must be avoided by women who are at increased risk of blood clotting, such as women with lupus who have phospholipid antibodies (including cardiolipin antibodies or lupus anticoagulant).
Individuals with SLE may improve prediction (prognosis) those with learning about the many aspects of the disease as well as strict monitoring of their health with their doctors.
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