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Types Of Lupus
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SLE;  Is the most serious of the lupus group of diseases;
Systemic lupus erythematosus:
 
Patients with SLE (female:male ratio is about 10:1) frequently present with polyarthritis - typically a peripheral polyarthritis with symmetric involvement of both small and large joints. The physician should question the patient in detail about symptoms that reflect multisystem involvement, particularly photosensitivity, unexplained rashes, malar rash, pleuritic chest pain, history of seizures, oral ulcers, hair loss, Raynaud's phenomenon, fevers and sweats.
 
Deformities including subluxation at the MCP joints, ulnar deviation, "swan neck" and boutonniere deformities (Jaccoud's arthropathy) may develop in about approximately 15% of patients with SLE, but these are not associated with joint erosion. (*polyarthritis--inflammation in more than 4 joints).

If, on the base of history and the physical examination SLE is suspected the physician should order an antinuclear antibody test;this is a useful screening test because a negative test result will virtually exclude SLE. If the test is positive and there is clinical suspicion of multisystem disease, the physician should consider further serologic tests and refer the patient to a rheumatologist.

For SLE patients without serious internal organ involvement hydroxychloroquine is the drug of choice because it has been shown to improve disease control, prevent flares and improve long-term outcomes. Arthritis and pleurisy respond to NSAIDs. Steroid medications may also be required in low and moderate doses either intermittently or continuously.

Immunosuppressant drugs are required for those with serious internal organ involvement (e.g., cerebritis or glomerulonephritis).

SLE can fluctuate between active period (flare ups and exacerbation),and minimal symptoms or no symptoms (remission). Other types of lupus are discoid lupus erythematosus (DLE) and subucutaneous lupus (SCLE). With these types of lupus, skin rashes and sun sensitivity are the main symptoms,and the internal organs are not attacked. Approximately 10 % of people diagnosed with these limited forms of lupus go on to develop SLE.

What are the warning signs of lupus?  Pain in the muscles and joints of the hands, arms, shoulders, feet, knees, hips or jaw. The pain may move from area to area and may cause the skin to feel hot, be red or swell. Fever and loss of appetite.Sudden and unexplained weight loss or gain.

Sometimes the rash is across the cheeks and bridge of the nose. This is called butterfly rash. Sometimes the rash is red,and scaly,and appears on the face,scalp,ears,arms,or chest. A milder form of lupus called discoid lupus  (DLE) causes this type of rash.

Small usually painless sores in the moist lining of the mouth or nose may appear. These are called mucosal ulcers. Changes in colour of the fingers when they are exposed to cold may be seen in some patients.

Sensivity to sunlight-increase in the number and severity of headaches-increase in loss of hair over the scalp-ongoing high blood pressure-swelling of feet and legs-chest pain when lying down,or taking deep breaths are symptoms seen in some patients.

SLE is a different disease for each person. Each patient has their own combination of symptoms as the disease targets the body's tissues and these symptoms range from mild to severe. If one experiences three or more of the warning signs,see a physician..

Some people with SLE may require no treatment if their symptoms are not severe and their disease is mild Treatment plans are based on the type and severity of symptoms which are individualized to meet each person's needs. No one test can diagnose lupus.

Although "lupus" is used as a broad term, there actually are several kinds of lupus:

  • Systemic lupus erythematosus (SLE) is the form of the disease that most people are referring to when they say "lupus." The word "systemic" means the disease can affect many parts of the body. The symptoms of SLE may be mild or serious. Although SLE usually first affects people between the ages of 15 and 45 years, it can occur in childhood or later in life as well. This booklet focuses on SLE.
  • Discoid lupus erythematosus refers to a skin disorder in which a red, raised rash appears on the face, scalp, or elsewhere. The raised areas may become thick and scaly and may cause scarring. The rash may last for days or years and may recur. A small percentage of people with discoid lupus have or develop SLE.
  • Drug-induced lupus refers to a form of lupus caused by specific medications. Symptoms are similar to those of SLE (arthritis, rash, fever, and chest pain) that typically go away when the drug is stopped.
  • Neonatal lupus is a rare form of lupus affecting newborn babies of women with SLE or certain other immune system disorders. At birth, the babies have a skin rash, liver abnormalities, or low blood counts, which entirely go away over several months. However, babies with neonatal lupus may have a serious heart defect. Physicians can now identify most at-risk mothers, allowing for prompt treatment of the infant at or before birth. Neonatal lupus is very rare, and most infants of mothers with SLE are entirely healthy.

Crossover,or overlap syndrome indicates features that overlap into another rheumatic disease. e.g., Rhupus (coined phrase) -overlap of RA and lupus.

Arthritis: This is one of the most common features in lupus. It occurs in the same pattern as rheumatoid arthrits-involving many joints,small and large,on both sides of the body,and particular the knucles of the fingers.

There is a couple of big differences from RA,however. The arthritis tends to be milder.reponds to treatment more easily and  does not  result in cartilage and bone damage. While the intense inflammation in the fingers may cause finger deformity, this is uncommon (less than 10 %). When it does happen,it's because of inflammatory weakening of the ligaments supporting the joints.

Joint pains (arthralgia) without obvious inflammation are as common as swollen joints. The tendons that anchors the kneecap to the upper shinbone may become weakened and even rupture.

Inflammation in the covering (the pleura) of the lungs (pleurisy),and inflammation of the lining of the heart (pericardium) is called pericarditis. The inflammation itself is usually without symptoms. When symptoms do occur,though,they can be very serious.

Like just about every other part of the body,if the kidneys are carefully studied in patients with lupus,some abnormality will usually be found. And as with just about every part of the body,these abnormalities can be very serious,but usually aren't.

Almost every part of the nervous system can be affected by lupus inflammation, when it occurs. The two most common problems,are headache, usually of  the migrane type,  and difficulties with ,memory,learning,and concentration. 

A number of other effects are not common,but do occur. These include coma, stroke seizures, and nerve damage. Psychiatric symptoms,in particular severe depression,may dominate in certain patients.

Sorting out these problems,trying to determine if they are caused by lupus itself,by the treatment of lupus,or some other factor,can be difficult.

If the patient has only a few symptoms-such as joint pain-and the ANA test is positive, a diagnosis of lupus is hard to make. Some patients need to be followed and tested for years before the picture becomes crystal-clear.

If the lupus is active,adequate rest is important-even to the point of taking time off from regular activities.

The Latin meaning for Lupus is "wolf" perhaps,because of it' many different symptoms. Some refer Lupus as a disease with "1000 faces". Lupus can be a life-threatening disease. RA also has mild,moderate,and severe types of cases.

Lupus strikes about one in two thousand people-fourteen to fifteen thousand Canadians -women eight to ten times more often than men. It usually appears in women between the ages of twenty and forty,although it can occur at any age in either gender and is equally common to both after age fifty. In severe cases it may cause death. In fact,as recently as the 1940s,only one patient in six survived three years after diagnosis.

Thanks to extensive clinical and basic science research,that last grim syatistic has been radically upgraded,to something like one fatality in ten cases a decade after diagnosis. by far,the majority of people with lupus live full,basically normal lives,as the disease and it's symptoms can usually be treated,as in RA.

People with lupus often suffer from a double affliction. There's the disease itself,but added to that is an emotionally corroding experience: the misunderstanding and skepticism,sometimes outright disbelief,that many people with lupus are subjected to by colleagues,family members,and friends-even some physicians. It's partly a visibility problem: How can you be sick,someone will ask,when you look perfectly healthy ? I am sure many RA patients have sililar experiences.

Such queries usually reflect the disease's own fickle mercies. Sometimes you're up,sometimes down,victim to a inconstant spectrum of ills. Lupus isn't a "true" form of arthritis;more accurately,it's a connective-tissue disease,though it's classified as a rheumatic disease because it's symptoms usually include joint pain and swelling. About half of all people with lupus do develop recognizable arthritis,though very few of them suffer the deformities associated with that disease.

There are still major questions concerning the cause,or causes,of lupus,and a cure is still,far off. (It's known that some people develop drug-induced lupus from taking medications for other conditions.) It iss certain,that lupus is an autoimmune disease,a disorder in which the body's own defenses turn on itself.

Furthermore,there appears to be an inherited predisposition for the disease,although the specific gene or genes involved have yet to be clearly defined. Current theory suggests that heredity alone can't explain why some people develop the disease while others don't. Some external trigger,perhaps a bacterium or virus,may be resposible for starting the disease process in genetically predisposed people. It could also be the result of one or more environmental factors,such as childbirth,hormonal changes,a traumatic injury,an infection-even sunlight-we still don't know for sure.

In some people with lupus, only one system of the body such as the skin or joints is affected. Other people experience symptoms in many parts of their body. Just how seriously a body system is affected also varies from person to person. Most commonly, joints and muscles are affected, causing arthritis and muscle pain. Skin rashes are quite common. The following systems in the body also can be affected by lupus.

  • Kidneys: Inflammation of the kidneys (nephritis) can impair their ability to get rid of waste products and other toxins from the body effectively. Because the kidneys are so important to overall health, lupus affecting the kidneys generally requires intensive drug treatment to prevent permanent damage. There is usually no pain associated with kidney involvement, although some patients may notice that their ankles swell. Most often the only indication of kidney disease is an abnormal urine or blood test.
  • Lungs: Some people with lupus develop pleuritis, an inflammation of the lining of the chest cavity that causes chest pain, particularly with breathing. Patients with lupus also may get pneumonia.
  • Central nervous system: In some patients, lupus affects the brain or central nervous system. This can cause headaches, dizziness, memory disturbances, vision problems, stroke, or changes in behavior.
  • Blood vessels: Blood vessels may become inflamed (vasculitis), affecting the way blood circulates through the body. The inflammation may be mild and may not require treatment or may be severe and require immediate attention.
  • Blood: People with lupus may develop anemia, leukopenia (a decreased number of white blood cells), or a decrease in the number of platelets (thrombocytopenia). Some people with lupus may have abnormalities that cause an increased risk for blood clots.
  • Heart: In some people with lupus, inflammation can occur in the heart itself (myocarditis and endocarditis) or the membrane that surrounds it (pericarditis), causing chest pains or other symptoms. Lupus can also increase the risk of atherosclerosis.

Diagnosing Lupus

Diagnosing lupus can be difficult. It may take months or even years for doctors to piece together the symptoms to diagnose this complex disease accurately. Making a correct diagnosis of lupus requires knowledge and awareness on the part of the doctor and good communication on the part of the patient. Giving the doctor a complete, accurate medical history (for example, what health problems you have had and for how long) is critical to the process of diagnosis. This information, along with a physical examination and the results of laboratory tests, helps the doctor consider other diseases that may mimic lupus, or determine if the patient truly has the disease. Reaching a diagnosis may take time and occur gradually as new symptoms appear.

No single test can determine whether a person has lupus, but several laboratory tests may help the doctor to make a diagnosis. The most useful tests identify certain autoantibodies often present in the blood of people with lupus. For example, the antinuclear antibody (ANA) test is commonly used to look for autoantibodies that react against components of the nucleus, or "command center," of the patient’s own cells. Most people with lupus test positive for ANA; however, there are a number of other causes of a positive ANA besides lupus, including infections, other rheumatic or immune diseases, and occasionally as a finding in normal healthy adults. The ANA test simply provides another clue for the doctor to consider in making a diagnosis. In addition, there are blood tests for individual types of autoantibodies that are more specific to people with lupus, although not all people with lupus test positive for these and not all people with these antibodies have lupus. These antibodies include anti-DNA, anti-Sm, anti-RNP, anti-Ro (SSA), and anti-La (SSB). The doctor may use these antibody tests to help make a diagnosis of lupus.

Some tests are used less frequently but may be helpful if the cause of a person’s symptoms remains unclear. The doctor may order a biopsy of the skin or kidneys if those body systems are affected. Some doctors may order a syphilis test or a test for anticardiolipin antibody. A positive test does not mean that a patient has syphilis; however, the presence of this antibody may increase the risk of blood clotting and can increase the risk of miscarriages in pregnant women with lupus. Again, all these tests merely serve as tools to give the doctor clues and information in making a diagnosis. The doctor will look at the entire picture--medical history, symptoms, and test results--to determine if a person has lupus.

Other laboratory tests are used to monitor the progress of the disease once it has been diagnosed. A complete blood count, urinalysis, blood chemistries, and erythrocyte sedimentation rate (ESR) test can provide valuable information. Another common test measures the blood level of a group of substances called complement. People with lupus often have increased ESRs and low complement levels, especially during flares of the disease.

 


 

What is Systemic Lupus Erythematosus ?

Systemic lupus erythematosus (also called SLE or lupus) causes a variety of problems. It may cause skin rashes, arthritis, anemia, seizures or psychiatric illness, and often affects internal organs including the kidneys, lungs and heart. Once a disease with high mortality, SLE is now considered a chronic disease. In 1954, survival after 4 years was 50%; today it is more than 97%.

CAUSE

SLE is a chronic inflammatory disorder resulting from an abnormality of the immune system, which normally functions to protect the body against cancers and invading infections. In SLE, the immune system is over-active and produces too many abnormal antibodies that react with the patient’s own tissues. The exact cause of lupus is not known, but heredity, environment and hormonal changes may be involved.

HEALTH IMPACT

  • Prevalence of SLE is 40 to 50 per 100,000.
  • It is more common in certain ethnic groups, particularly among blacks.
  • More than 85 percent of lupus patients are women.

DIAGNOSIS

Because of its wide variety of symptoms, diagnosis is often difficult and requires a high degree of awareness among physicians. Typical features of SLE include:

  • A butterfly-shaped rash over the cheeks
  • A skin rash appearing in areas exposed to the sun
  • Sores in the mouth and nose
  • Arthritis involving one or more joints
  • Kidney inflammation
  • Nervous system disorders including seizures, mental disorders and strokes

Fever, weight loss, hair loss, poor circulation in the fingers and toes, chest pain when taking deep breaths (pleurisy) and abdominal pain are often seen. Laboratory studies are crucial to diagnosing SLE. In particular, the antinuclear antibody (ANA) test is almost always positive in SLE. A precise diagnosis is often appropriately delayed because the disease may evolve gradually.

TREATMENT

Treatment for SLE depends on the clinical problems present and whether the disease is active or not at a given time. Earlier and more accurate diagnosis, better understanding of the immune abnormalities in SLE, and treatment studies have all contributed to improved treatment of patients with SLE.

Regular medical evaluation is important to monitor SLE. Drug treatment must be individualized for each patient, depending on the particular problems and their severity. For mild inflammation, nonsterodial anti-inflammatory medications are helpful. Corticosteroids, the single most important drugs to treat SLE, must be used judiciously. Bone protection is important when steroids are used. Anti-malarials such as hydroxychloroquine reduce SLE activity and are helpful for the skin and joints. More severe SLE requires immunosuppressive drugs such as azathioprine and cyclophosphamide. The disease often enters quiet periods with little or no activity (remission), during which medications can be reduced and occasionally discontinued

That unknown trigger is just part of the reason physicians are at such pains to diagnose the condition. then there's the fact that no two ptient's suffer,or present with symptoms,in quite the same way. Symptoms can include any thing from hair and weight loss and rashes to ulcerated mouth sores and throat and facial swelling (which may indicate kidney failure). Some people develop mainly joint problems. And any patient can get any combination of the full menu.

Diagnosis begins with a patient's history. A family history of lupus or another immune disorder,such as RA,is a strong clue. The physician then performs a physical examination to confirm suspecions raised by the history. Joint inflammation,for example,would suggest arthritis,but if there's a rash that flares from exposure to the sun,or other rashes on the body consistent with lupus the diagnosis is clearer.

There are a number of other techniques a physician can perform to confirm the diagnosis,including tests for inflammation around the lungs and heart. There are also lab tests to identify lupus-associated immunological dysfunctions,including tests for FANA (fluorescent antinuclear antibody),anti-DNA,and anti-Sm (a substance found in cell nucleii that was named after a patient-named Smith- in whom the first "anti-Smith" antibody was found).