Monday, September 28, 2009

Lupus .. (Systemic Lupus Erythematosus)

Lupus Overview
Systemic lupus erythematosus (lupus, SLE) is an autoimmune disease in which a person's immune system attacks various organs or cells of the body causing damage and dysfunction. Lupus is called a multisystem disease because it can affect many different tissues and organs in the body. Some patients with lupus have a very mild condition, which can be treated with simple medication , whereas others can have serious, life-threatening complications. Lupus is more common in women than men, and its peak incidence is after puberty -the reason for this is unknown.

While lupus is a chronic illness, it is characterized by periods when the disease activity is minimal or absent (remission) and when it is active (relapse or flare). The outlook for patients with SLE today is much better than years ago because of greater awareness and better tests leading to earlier diagnosis and treatment and more effective and safer medications.

Lupus Causes Genetic link
As with other autoimmune diseases, people with lupus share some type of common genetic link. An identical twin has a 3- to 10-fold greater risk of getting lupus than a nonidentical twin. Also, first-degree relatives (mother, father, brother, sister) of people with lupus have an 8- to 9-fold increased risk of having lupus than the general public.

Environmental factors
Although an identical twin is much more likely to have lupus if her identical sibling has lupus, the likelihood of developing the disease in the unaffected twin is not 100%. Despite the nearly identical genetic makeup of identical twins, the probability of the unaffected twin developing the disease if the other twin has it is around 30-50% or less. This implies that environmental factors may help determine whether or not the disease develops in a person. Outside of random occurrences of lupus, certain drugs, toxins, and diets have been linked in its development. Sun exposure (ultraviolet light) is a known environmental agent that can worsen rashes of lupus patients and sometimes trigger a flare of the entire disease.

Reversible drug-induced lupus
In the past, the drugs most frequently responsible for drug-induced lupus are procainamide (Procanbid), hydralazine (Apresoline), and isoniazid (Laniazid). However, newer medications have been associated with drug-induced lupus, such as the new biological agents used to treat rheumatoid arthritis, etanercept (Enbrel), infliximab (Remicade), adalimumab (Humira). Generally, lupus that is caused by a drug exposure goes away once the drug is stopped.

Association with pregnancy and menstruation
Many women with lupus note that symptoms may be worse after ovulation and better at the beginning of the menstrual period. Estrogen has been implicated in making the condition worse and this problem is currently being studied. For this reason, women with lupus who are on birth control medications are encouraged to take those that contain little or no estrogen.

Lupus Symptoms
At the onset of lupus, the symptoms are commonly very general and therefore make diagnosing the disease difficult. The most common initial complaints are fatigue, fever, and muscle and joint pain. This is called a "flulike syndrome."

· Fatigue is the most common and bothersome complaint. It is also often the only symptom that remains after treatment of acute flares. A flare in lupus is an acute increase in symptoms.
· Fever during lupus flares is usually low-grade, rarely exceeding 102°F. A temperature greater than this should stimulate a search for an infection as the source of the fever. However, any fever in lupus should be considered an infection until proven otherwise.
· Muscle pain (myalgia) and joint pain (arthralgia) without or with joint swelling (arthritis) are very common with the new onset of lupus and with subsequent flares.
Although lupus is a multisystem disease, certain organs are affected more commonly than others:
· Musculoskeletal system: Joint pains are more common than arthritis in people with lupus. The arthritis of lupus is usually found on both sides of the body and does not cause damage to the joints. The most frequently involved joints are those of the hand, knees, and wrists. People with lupus, especially those needing high doses of corticosteroids (steroids, prednisone), can suffer from a certain type of low blood flow injury to a joint causing death of the bone in the joint. This is called avascular necrosis and occurs most commonly in the hips and knees. The muscles themselves can sometimes become inflamed and very painful contributing to weakness and fatigue.

· Skin and hair: The skin is involved in more than 90% of people with lupus. Skin symptoms are more common in whites than in African Americans. While the classic lupus rash is a redness on the cheeks (malar blush) often brought on by sun exposure, many different types of rashes can be seen in SLE. Discoid lupus with the red skin patches on the skin and scaliness is a special characteristic rash that can lead to scarring. It usually occurs on the face and scalp and can lead to loss of scalp hair (alopecia). It is more common in African Americans with lupus. Occasionally, discoid lupus can occur as an isolated skin condition without systemic disease. Hair loss can occur with flares of SLE even without skin rashes in the scalp. In this situation, the hair regrows after the flare is treated. Hair loss can also occur with immunosuppressive medications.

Kidney system: The kidney is involved clinically in more than half of all people with lupus. Severe kidney disease often requires immunosuppressive therapy. All patients with newly diagnosed SLE should have the urine checked for blood and protein because kidney inflammation can be silent in the early stages

· Heart and blood vessels: Inflammation of the sac holding the heart (pericarditis) is the most common form of heart problem in people with lupus. This causes chest pain and can mimic a heart attack. Also, growths (vegetations) can form on heart valves causing heart problems. Hardening of the arteries (atherosclerosis) can lead to angina and heart attacks in lupus patients who have required long-term prednisone therapy for severe disease. In some people with lupus, the arterial blood supply to the hand can experience intermittent interruptions due to spasm of the artery. This causes whiteness and blueness in the fingers and is called Raynaud syndrome. It is brought on by emotional events, pain, or cold temperatures.

· Nervous system: Serious brain and nerve problems and acute psychiatric syndromes occur in about 15% of patients with lupus. Potential disorders include seizures, nerve paralysis, severe depression, psychosis, and strokes. Spinal cord inflammation in lupus is rare but can cause paralysis. Depression is common in SLE. Sometimes it is directly related to active disease and sometimes to emotional difficulties in coping with a chronic illness or to the medications used to treat it, especially high doses of prednisone.

· Lungs: More than 50% of people with lupus have some sort of lung disease. Inflammation of the lining of the lung (pleurisy) is the most common problem. This can lead to chest pain and shortness of breath and can be confused with clots in the lung or lung infection (pneumonia). Collections of water in the space between the lung and the chest wall occur as well (called pleural effusions). Pneumonia can occur in lupus patients who are taking immunosuppressive medications.

· Blood and lymph system: About half of people with lupus have anemia (low red blood cell counts), and up to half can have thrombocytopenia (low platelet counts) and leukopenia (low white blood cell counts). Low platelet counts can lead to bleeding and bruising in the skin and if severe, it can cause internal bleeding. Some lupus patients are predisposed to developing blood clots in veins (leading to phlebitis) or arteries (leading to strokes or other problems). This is most likely to occur in patients who have certain autoantibodies in their blood called antiphospholipid antibodies. Patients with these clinical problems and these antibodies may need to take blood thinners (anticoagulants) for prolonged periods of time. Women with these antibodies can also suffer from a high frequency of spontaneous miscarriages.

· Stomach, intestines, liver, and associated organs: Many patients with lupus develop painless ulcers in the mouth and nose at some point in their disease. Abdominal pain in lupus can be due to inflammation of the lining of the abdomen, infection of the intestines, low blood flow to the intestines caused by a clot, or inflammation of the vessels flowing to the intestines. If the person has a lot of free-floating fluid in the abdomen, this fluid may also become infected causing severe pain. The liver is rarely involved.
· Eyes: The eyes are rarely involved in lupus, except for the retina. People with lupus often have to be screened by an ophthalmologist if they are taking the antimalarial drugs chloroquine or hydroxychloroquine.

When to Seek Medical Care
· High fever
· Unusual headache
· Blood in the urine
· Chest pain
· Shortness of breath
· Swelling of the legs
· Weakness of the face, arms, or legs, on one side
· Unusual abdominal pain
· Unusual joint pain
· Recurrent pregnancy loss (miscarriages)
· Visual disturbances

When to go to the hospital
· Fever greater than 102°F
· Rapidly decreasing urine volume
· Chest pain
· Sudden onset or unusual shortness of breath
· Sudden onset of weakness
· Severe headache
· Acute visual changes
· Sudden onset of abdominal pain
· Inability to bear weight or move a swollen joint due to severe pain
· Rapid swelling of one or more extremities (arms, legs, hands, or feet)

Seal’s facial scars are the result of discoid lupus
Exams and Tests Medical evaluation in the emergency department
The nature of the medical evaluation in an emergency department depends on the person's complaint or problem. The goal of the emergency physician is to decide whether there are acute concerns on top of a chronic disease such as lupus. For someone without a known diagnosis of lupus, the doctor will evaluate but likely never provide a primary diagnosis of lupus unless there are clear and unmistakable features. Evaluation of chronic (not acute) diseases is done in a medical office setting.

The vast majority of blood tests looking for specific markers for lupus do not return results for several days, so definitive blood tests do not occur on the spot. However, certain tests to assess the possibility of organ damage including some blood and urine tests, imaging studies, and heart tracings will allow the doctor to assess for acute disease if someone with lupus seeks treatment in an emergency department.

The job of the emergency doctor is to address, evaluate, and treat acute issues and then refer the person to more appropriate specialists for further assessment and evaluation.

Criteria for diagnosing lupus
The diagnosis of lupus is a clinical one made by observing symptoms. Lab tests provide only a part of the picture. The American College of Rheumatology has designated 11 criteria for classification.

To be classified as having lupus, a person must have 4 or more of these criteria:
· Malar rash: This is a "butterfly-shaped" red rash over the cheeks below the eyes. It may be a flat or a raised rash.
· Discoid rash: These are red, raised patches with scaling of the overlying skin. A subgroup of patients have "discoid lupus" with only skin involvement and do not have systemic lupus erythematosus. All patients with discoid lupus should be screened for systemic involvement.

· Photosensitivity: A rash develops in response to sun exposure. This is not to be confused with heat rash that develops in body folds or moist areas of the body with exposure to heat.
· Oral ulcers: Painless sores in the nose or mouth need to be observed and documented by a doctor.

· Arthritis: The arthritis of lupus usually does not cause deformities of the joints. Swelling and tenderness must be present.
· Serositis: This refers to an inflammation of various "sacs" or membranes that cover the lung, cover the heart, and line the abdomen. Inflammation of these tissues causes severe discomfort in the areas affected.
· Kidney disease: There is persistent loss of protein in the urine, or a microscopic analysis of the urine demonstrates inflammation of the kidneys.
· Neurological disorder: This can present as seizures or as a primary psychiatric disorder.
· Blood disorder: Low blood counts of various blood components are known to occur.
· Immunologic disorder: This requires special laboratory testing for specific markers of disease in lupus. These tests include antibodies to DNA, a nuclear protein (Sm), or phospholipids (which includes the falsely positive test result for syphilis). The presence of these and other antibodies that can react with the body's own tissues is why lupus is called an autoimmune disease.
· Positive antinuclear antibody: A more general marker in the blood for the presence of an autoimmune disease, these "ANA" levels increase with age, thereby somewhat increasing the rate of an incorrectly positive test as a person gets older. The ANA test is most useful when the result is negative, which essentially rules out the diagnosis of SLE, since up to 98% of people with lupus have a positive ANA test result.

HeLa Cell Histones

Laboratory Tests for Lupus
Many different laboratory tests are used to detect physical changes or conditions in your body that can occur with lupus. Each test result adds more information to the picture your doctor is forming of your illness.

Routine Blood Tests
Usually your doctor will first request a complete blood count (CBC). Your blood is made up of red blood cells, white blood cells, platelets, and serum. The complete blood count measures the levels of each. In cases of lupus, these blood tests may reveal low numbers.
· Red blood cells carry oxygen to all parts of the body.
· White blood cells (lymphocytes and others) help the immune system to protect the body against foreign invaders. The white blood cells called T cells and macrophages are directly involved in this protection. Other white blood cells called B cells are indirectly involved, by producing antibodies to the foreign substances.

· Platelets form in bone marrow; they go to the site of a wound to begin the blood-clotting process.
· Blood serum is the fluid portion of whole blood from which certain substances in the clotting of blood have been removed.

Urine Tests
Because your body’s waste is processed by the kidneys, testing a sample of urine (called a 'spot urine' test) can reveal any problems with the way your kidneys are functioning. Lupus can attack the kidneys without any warning signs, so these tests are very important. The most common urine tests look for cell casts (bits of cells that normally would be removed when your blood is filtered through your kidneys), and proteinuria (protein being spilled into your body because your kidneys are not filtering the waste properly). A collection of your urine over a 24-hour period can also give important information.

The antibodies your body makes against its own normal cells and tissues play a large role in lupus. Many of these antibodies are found in a panel, or group, of tests that are ordered at the same time. The test you will hear most about is called the ANA test. This is not a specific test for lupus, however.

Antinuclear antibodies (ANA) are antibodies that connect, or bind, to the nucleus -- the "command center" -- of the cell. This process damages, and can destroy, the cells. The ANA blood test is a sensitive test for lupus, since these antibodies are found in 97 percent of people with the disease. When three or more typical features of lupus are present -- such as involvement of the skin, joints, kidneys, lungs, heart, blood, or nervous system -- a positive ANA test will confirm a diagnosis of lupus. However, a positive ANA test result does not always mean you have lupus. The ANA can be positive in people with other illnesses, or positive in people with no illness. The ANA can also change from positive to negative, or negative to positive, in the same person. Still, lupus is usually the diagnosis when these antinuclear antibodies are found in your blood.
In addition to the ANA, doctors trying to diagnose lupus often look for the following specific antibodies.

Antibodies to double-stranded DNA (anti-dsDNA) are antibodies that attack the DNA -- the genetic material -- inside the cell nucleus. Anti-dsDNA antibodies are found in half of the people with lupus, but lupus can still be present even if these antibodies are not detected.

Antibodies to histone -- a protein that surrounds the DNA molecule -- are sometimes found in people with systemic lupus but are more often seen in people with drug-induced lupus. This form of lupus is caused by certain medications, and usually goes away after the medication is stopped.

Antibodies to phospholipids (aPLs) can cause narrowing of blood vessels, leading to blood clots in the legs or lungs, stroke, heart attack, or miscarriage. The most commonly measured aPLs are lupus anticoagulant, anticardiolipin antibody, and anti-beta2 glycoprotein I. Nearly 30 percent of people with lupus will test positive for antiphospholipid antibodies. Phospholipids found in lupus are also found in syphilis, and the blood test cannot always tell the difference between the two diseases. A positive result to a syphilis test does not mean that you have or ever had syphilis. Approximately 20 percent of those with lupus will have a false-positive syphilis test result.

Antibodies to Ro/SS-A and La/SS-B (Ro and La are the names of proteins in the cell nucleus) are often found in people with Sjögren’s syndrome. Anti-Ro antibodies in particular will be found in people with a form of cutaneous (skin) lupus which causes a rash that is very sun-sensitive. It is especially important for your doctor to look for the Ro and La antibodies if you are pregnant, as both autoantibodies can cross the placenta and can cause neonatal lupus in the infant. Neonatal lupus is rare and not usually dangerous, but it can be serious in some cases.

Antibodies to Sm target Sm proteins in the cell nucleus. Found in 30-40 percent of people with lupus, the presence of this antibody almost always means that you have lupus.
Antibodies to RNP target ribonucleoproteins, which help to control chemical activities of the cells. Anti-RNPs are found in many autoimmune conditions and will be at very high levels in people whose symptoms combine features of several diseases, including lupus.

Other Blood Tests
Some blood tests measure levels of proteins that are not antibodies. The levels of these proteins can alert your doctor that there is inflammation somewhere in your body.
Complement is the name of a group of proteins that protect the body against infections. They work by strengthening the body’s immune reactions. Complement proteins are used up by the inflammation caused by lupus, which is why people with inflammation due to active lupus often have low complement levels. There are nine protein groups of complement, so complement is identified by the letter C and the numbers 1 through 9. The most common complement tests are CH50, C3, and C4. CH50 measures the overall function of complement in the blood. Low levels of C3 or C4 may indicate active lupus.

C-reactive protein (CRP) is a protein produced by the liver, and high levels of CRP in your blood may mean you have inflammation due to lupus.
Erythrocyte sedimentation rate (ESR or "sed" rate) is another test for inflammation. It measures the amount of a protein that makes the red blood cells clump together. The sed rate is usually high in people with active lupus, but it can also be high due to other reasons, such as an infection.

Blood Clotting Time Tests
The rate at which your blood begins to clot is important: if it clots too easily, a blood clot, called a thrombus, can break free and travel through the body. Blood clots can cause damage such as a stroke or miscarriage. If your blood does not clot quickly enough, you could be at risk for excessive bleeding if you are injured.
Prothrombin time (PT) measures blood clotting and can show whether you may be at risk for not clotting quickly enough at the site of a wound.
Partial thromboplastin time (PTT) also measures how long it takes for your blood to begin to clot.

Modified Russell viper venom time (RVVT), platelet neutralization procedure (PNP), and kaolin clotting time (KCT) are other, more sensitive blood-clotting time tests.

Tissue Biopsies
A biopsy procedure involves removal of a small bit of tissue which the doctor then examines under a microscope. Almost any tissue can be biopsied.
· The skin and kidney are the most common sites biopsied in someone who may have lupus.
· The results of the biopsy can show the amount of inflammation and any damage being done to the tissue.
· Further tests can be performed on the tissue sample to look for autoimmune antibodies and to determine whether the problem is due to lupus or is caused by some other factor, such as infection or medication.

Lupus Treatment Self-Care at Home
Home care for lupus generally involves taking the prescribed medications and adhering to good practices such as using sunscreen because there is often a history of skin sensitivity to sunlight.
· People with sun-induced rashes should always wear a high SPF lotion that blocks both UVA and UVB types of ultraviolet light.
· Those taking oral steroid therapy or immune suppressing agents should be vigilant if a fever develops, since fever can occur with lupus flares or with a superimposed problem, especially infection.
· A combination of rest, especially during flares, and exercise for joints and muscles is important and should be supervised by the treating physician and physical therapists.

Medical Treatment
For a person known to have lupus and already taking medications for it, every effort will be made in the emergency department to avoid the addition of potentially dangerous medications with significant side effects.

· The use of ibuprofen (Motrin, Advil) and medications like ibuprofen in treating lupus requires some caution. Ibuprofen and similar drugs can harm kidney function, especially in people who already have kidney problems. In addition, ibuprofen and related agents can rarely cause inflammation of the lining of the brain resulting in a severe headache.

· Many people with lupus can experience significant relief of their symptoms without the use of steroids or other immune-suppressing agents (such as azathioprine or cyclophosphamide).

However, certain acute complications (such as acute kidney failure) caused by lupus may require high doses of oral or intravenous steroids along with other immune-suppressive drugs. Some people will require long-term treatment with steroids and immune-suppressing agents.

· Antimalarial drugs such as hydroxychloroquine and chloroquine are excellent alternatives for people with lupus who do not respond well to ibuprofen or aspirin. Many people on antimalarial drugs experience significant relief of their symptoms, especially rashes, fatigue, and joint and muscle pains. Hydroxychloroquine has been shown to decrease the frequency of flares in patients with systemic lupus erythematosus. Based on these data, it is widely believed that all patients should be treated with hydroxychloroquine indefinitely, unless they develop adverse effects. However, with antimalarial use, careful periodic evaluation of the eyes is required to prevent serious complications.

· For people with sun-sensitive lupus rashes, appropriate use of ultraviolet-blocking sunscreens and protective clothing is critical. Heat, infrared light, and, rarely, fluorescent light can also bring on flares. Topical steroid creams are also helpful for lupus-associated rashes, once they develop. A doctor should closely monitor extended use of steroid creams, especially on the face and covered areas.

· Treatment of seizures or psychiatric disturbances usually involves therapy directed at the type of disturbance itself (the use of anticonvulsants for seizures, for example, and the use of antidepressants for severe depression).

· Steroid use is associated with a number of complications including psychiatric disturbances, increased susceptibility to infection, fragile bones, cataract formation, diabetes and worsening of existing diabetes, high blood pressure, thinning of the skin, puffiness of the face, and avascular necrosis. Steroids are often reserved for lupus patients with serious organ involvement or lupus that does not respond to other medications.

o An important side effect of steroids and other immune-suppressing agents is an increase in the susceptibility to dangerous infections.
o In pregnancy, the preferred steroid is prednisone because it crosses into the fetus much less than other steroid agents.
o Steroids should not be stopped abruptly if you have been taking them for more than several months. Your health care provider will direct you how to taper the medicine.
o If blood clots form spontaneously in the body, treatment with an agent that prevents clot formation is critical. For this reason, use of heparin or warfarin (Coumadin) is advised. In pregnancy, heparin is the agent of choice because of the adverse fetal effects of warfarin.
Next Steps Outlook
The prognosis varies depending on whether there is serious organ inflammation (for example kidney or brain involvement).

Many lupus patients have very limited disease and live relatively normal lives with minimal problems. Others have multi-organ involvement with kidney failure, heart attacks, and strokes. The diversity of outcomes reflects the diversity of the disease.

With respect to fertility, women with lupus are just as capable of becoming pregnant and having children as the general population. However, there is a much higher occurrence of complications in pregnancy-especially if the kidneys are involved.

Women whose lupus has been inactive for 6-12 months are more likely to have a successful pregnancy. In addition, antibodies formed in the mother that are transferred from mother to fetus can occasionally affect the infant, leading to rashes, low blood counts, or more seriously a slow heart rate due to complete heart block (neonatal lupus). For these reasons, all women with lupus who are or who desire to become pregnant should consult with their treating rheumatologist or other treating physicians and should be referred for "high-risk" obstetric care.

Chinese medical journals have relayed information about lupus treatment throughout the 20th Century and it remains a subject of intense interest. In most cases, lupus is treated in China with corticosteroids; in addition, herbal therapies are frequently employed as an adjunct to steroids. The modern Chinese literature focuses on two primary means of treating lupus with herbs: using antitoxin herbs (usually in conjunction with herbs that vitalize blood circulation) and using ching-hao or its active components.

he basic herb formula was composed of the toxin-cleaning, stasis-resolving, and yin-nourishing agents (the latter help control the heat syndrome)
The nephrotic syndrome that often accompanies lupus after several years of flare-ups is one of the life-threatening complications, though modern drug therapy and renal transplant technology can prevent loss of life. the syndrome is usually marked by a large quantity of albumin (protein) in the urine, with corresponding decline of serum albumin, and with some edema. From the perspective of traditional Chinese medicine, most of these patients suffer from qi and/or yang deficiency of the spleen and kidney, often with complications of blood stasis.

We do have the following herbs; which are beneficial to treat patient with systemic or discoid lupus.

Artemisia leaf, isatis leaf, oldenlandia, red peony, moutan bark, cimicifuga, rehmannia, ophiopogon and more

Please ask for it, we deliver our herbs all over the world