What is lupus? What are the types of lupus?

Lupus is an autoimmune disease characterized by acute and chronic inflammation of various tissues of the body. Autoimmune diseases are illnesses that occur when the body's tissues are attacked by its own immune system. The immune system is a complex system within the body that is designed to fight infectious agents, such as bacteria and other foreign microbes. One of the ways that the immune system fights infections is by producing antibodies that bind to the microbes. People with lupus produce abnormal antibodies in their blood that target tissues within their own body rather than foreign infectious agents. Because the antibodies and accompanying cells of inflammation can affect tissues anywhere in the body, lupus has the potential to affect a variety of areas. Sometimes lupus can cause disease of the skin, heart, lungs, kidneys, joints, and/or nervous system. When only the skin is involved, the condition is called lupus dermatitis or cutaneous lupus erythematosus. A form of lupus dermatitis that can be isolated to the skin, without internal disease, is called discoid lupus. When internal organs are involved, the condition is referred to as systemic lupus erythematosus (SLE). Both discoid and systemic lupus are more common in women than men (about eight times more common). The disease can affect all ages but most commonly begins from 20-45 years of age. Statistics demonstrate that lupus is somewhat more frequent in African Americans and people of Chinese and Japanese descent. What causes lupus? Is it hereditary? The precise reason for the abnormal autoimmunity that causes lupus is not known. Inherited genes, viruses, ultraviolet light, and certain medications may all play some role. Genetic factors increase the tendency of developing autoimmune diseases, and autoimmune diseases such as lupus, rheumatoid arthritis, and autoimmune thyroid disorders are more common among relatives of people 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, symptoms of lupus can be precipitated or aggravated by only a brief period of sun exposure. It also is known that some women with SLE can experience worsening of their symptoms prior to their menstrual periods. This phenomenon, together with the female predominance of SLE, suggests that female hormones play an important role in the expression of SLE. This hormonal relationship is an active area of ongoing study by scientists. More recently, research has demonstrated evidence that a key enzyme's failure to dispose of dying cells may contribute the development of SLE. The enzyme, DNase1, normally eliminates what is called "garbage DNA" and other cellular debris by chopping them into tiny fragments for easier disposal. 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 could disrupt the body's cellular waste disposal may be involved in the initiation of SLE. hat are the symptoms and signs of lupus? People with SLE can develop different combinations of symptoms and organ involvement. Common complaints and symptoms include fatigue, low-grade fever, loss of appetite,

muscle aches, arthritis, ulcers of the mouth and nose, facial rash ("butterfly rash"), unusual sensitivity to sunlight (photosensitivity), inflammation of the lining that surrounds the lungs (pleuritis) and the heart (pericarditis), and poor circulation to the fingers and toes with cold exposure (Raynaud's phenomenon). Complications of organ involvement can lead to further symptoms that depend on the organ affected and severity of the disease. Skin manifestations are frequent in lupus and can sometimes lead to scarring. In discoid lupus, only the skin is typically involved. The skin rash in discoid lupus often is found on the face and scalp. It usually is red and may have raised borders. Discoid lupus rashes are usually painless and do not itch, but scarring can cause permanent hair loss (alopecia). Over time, 5%-10% of those with discoid lupus may develop SLE. Over half of the people with SLE develop a characteristic red, flat facial rash over the bridge of their nose. Because of its shape, it is frequently referred to as the "butterfly rash" of SLE. The rash is painless and does not itch. The facial rash, along with inflammation in other organs, can be precipitated or worsened by exposure to sunlight, a condition called photosensitivity. This photosensitivity can be accompanied by worsening of inflammation throughout the body, called a "flare" of the disease.

Typically, with treatment, this rash can heal without permanent scarring. Most people with SLE will develop arthritis during the course of their illness. Arthritis in SLE commonly involves swelling, pain, stiffness, and even deformity of the small joints of the hands, wrists, and feet. Sometimes, the arthritis of SLE can mimic that of rheumatoid arthritis (another autoimmune disease). More serious organ involvement with inflammation occurs in the brain, liver, and kidneys. White blood cells and blood-clotting factors also can be characteristically decreased in SLE, known as leukopenia (leucopenia) and thrombocytopenia, respectively. Leukopenia can increase the risk of infection and thrombocytopenia can increase the risk of bleeding.

Inflammation of muscles (myositis) can cause muscle pain and weakness. This can lead to elevations of muscle enzyme levels in the blood. Inflammation of blood vessels (vasculitis) that supply oxygen to tissues can cause isolated injury to a nerve, the skin, or an internal organ. The blood vessels are composed of arteries that pass oxygen-rich blood to the tissues of the body and veins that return oxygen-depleted blood from the tissues to the lungs. Vasculitis is characterized by inflammation with damage to the walls of various blood vessels. The damage blocks the circulation of blood through the vessels and can cause injury to the tissues that are supplied with oxygen by these vessels. Inflammation of the lining of the lungs (pleuritis) and of the heart (pericarditis) can cause sharp chest pain. The chest pain is aggravated by coughing, deep breathing, and certain changes in body position. 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 due to coronary artery disease. Kidney inflammation in SLE can cause leakage of protein into the urine, fluid retention, high blood pressure, and even kidney failure. This can lead to further fatigue and swelling of the legs and feet. With kidney failure, machines are needed to cleanse the blood of accumulated waste products in a process called dialysis. Involvement of the brain can cause personality changes, thought disorders (psychosis), seizures, and even coma. Damage to nerves can cause numbness, tingling, and weakness of the involved body parts or extremities. Brain involvement is referred to as lupus cerebritis. Many people with SLE experience hair loss (alopecia). Often, this occurs simultaneously with an increase in the activity of their disease. The hair loss can be patchy or diffuse and appear to be more like hair thinning. Some people with SLE have Raynaud's phenomenon. In this condition, the blood supply to the fingers and/or toes becomes compromised upon exposure to cold, causing blanching, whitish and/or bluish discoloration, and pain and numbness in the exposed fingers and toes. HOW IS IT DIAGNOSED? The 11 criteria used for diagnosing systemic lupus erythematosus are

malar (over the cheeks of the face) "butterfly" rash, discoid skin rash (patchy redness with hyperpigmentation and hypopigmentation that can cause scarring), photosensitivity (skin rash in reaction to sunlight [ultraviolet light] exposure), mucous membrane ulcers (spontaneous ulcers of the lining of the mouth, nose, or throat), arthritis (two or more swollen, tender joints of the extremities), pleuritis or pericarditis (inflammation of the lining tissue around the heart or lungs, usually associated with chest pain upon breathing or changes of body position),

kidney abnormalities (abnormal amounts of urine protein or clumps of cellular elements called casts detectable with a urinalysis), brain irritation (manifested by seizures [convulsions] and/or psychosis), blood-count abnormalities (low counts of white or red blood cells, or platelets, on routine blood testing), immunologic disorder (abnormal immune tests include anti-DNA or anti-Sm [Smith] antibodies, falsely positive blood test for syphilis, anticardiolipin antibodies, lupus anticoagulant, or positive LE prep test), antinuclear antibody (positive ANA antibody testing [antinuclear antibodies in the blood]).

In addition to the 11 criteria, other tests can be helpful in evaluating people with SLE to determine the severity of organ involvement. These include routine testing of the blood to detect inflammation (for example, tests called the sedimentation rate and C-reactive protein), blood-chemistry testing, 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. The appropriate testing procedures are selected for the patient individually by the doctor. Systemic Lupus Erythematosus At A Glance

Systemic lupus erythematosus (SLE) is an autoimmune disease. SLE is characterized by the production of unusual antibodies in the blood. SLE is eight times more common in women than men. The cause(s) of SLE is (are) unknown, however, heredity, viruses, ultraviolet light, and drugs all may play some role. Up to 10% of people with lupus isolated to the skin will develop the systemic form of lupus (SLE). Eleven criteria help doctors to diagnose SLE. Treatment of SLE is directed toward decreasing inflammation and/or the level of autoimmune activity. People with SLE can prevent "flares" of disease by avoiding sun exposure and not abruptly discontinuing medications and monitoring their condition with their doctor.

A Lupus Widower Laments to Friends By Mr. D.R. Medical Editor: William C. Shiel Jr., MD, FACP, FACR So many people have had comments like, "I had no idea Susan was so sick." That was because Susan did not want anybody to know. Because lupus is such a crafty disease and flies under the general public's radar, this is a good opportunity to clear things up. Lupus is an autoimmune disease, not an infectious disease like HIV. People with lupus have an overactive immune system. The body's defenses actually attack healthy tissue. This has been happening to Susan for 30 years or more. She was diagnosed around 1990, but she had symptoms way before then. Originally, the disease would manifest itself as flu-like symptoms for about three weeks out of every three months, with fatigue extending a month after every flare-up. In 2000, lupus attacked her central nervous system and involved her brain. The original result of this new development was pain. From late in January 2000 until the day before she died, she was in pain. On the scale of 1 to 10, there was no day that she didn't feel pain on an 8 to 10 level. (With 10 being allencompassing.) Lupus attacked her lungs, causing shortness of breath, sleeping problems, and more pain in the form of pleurisy. In addition to Hashimoto's thyroiditis (causing

cold extremities) and Sjogren's syndrome (extremely dry eyes) and a few more isms that I have forgotten, life was getting difficult. Around 2003, it was emerging that her brain was more affected by the central nervous system lupus. As much as they tried, there was nothing that her doctors could do about this. Motor coordination, balance, and some cognitive skills were diminishing. It was at that time she decided to retire from teaching college and we planned to change our lifestyle. Her condition continued to worsen, and about two years ago, we decided that a lifestyle change was not to be and we vowed to just have fun every day. Also, it was time to tell our children that lupus was slowly killing Susan. Shortly afterward, she gave me permission to mildly explain her condition to a few friends. Some of you may recall those discussions. Earlier this year, we took her to the hospital. It was her ninth visit in less than four years. She was diagnosed with meningitis. It was the bacterial kind caused by listeriosis bacteriaa nasty bug, one of the doctors told me. Her condition at the time was described as grave. After a week, she was showing improvement, and 11 days after admission, the doctors took the breathing tube out. When I could go in and see her, I was excited to see her face without the tape and tubes, and I told her that I missed that face. To my surprise, she looked up and said, "I miss you, too." Those were her last words to me. While I imagined another week in the hospital and then a return home, the doctors cautioned me to think months and years for recovery and then never a full recovery. Two days later, she experienced convulsions and got bile into her lungs. She was without oxygen for an unspecified amount of time. The tube was back in, and her brain test was showing abnormal brain activity. She had internal bleeding and would need an operation to correct it. Shortly after, she had two strokes that would require brain surgery. One doctor took me aside and explained that the Susan I saw in bed at that moment was all the Susan I was ever likely to get. And the pain seemed to get worse for her. Nearly four weeks after admission, we put her on comfort medication and removed most of her tubes. What the doctors thought might take 20 minutes ended up taking 20 days. As the days wore on, Susan became more and more beautiful. The steroids wore off and her face looked as it did so long ago. The bruises from the tape healed and she looked as if she was 30. A month and a half after admission, she quietly slipped away at about 10:15 in the morning, with her daughter, son, and me at her side.

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GATAB, Krystal A. BSU- SNIII; Group H