What is Lupus? Systemic Lupus Erythematosus (SLE)

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Systemic Lupus Erythematosus (SLE) a chronic inflammatory disease that can affect various parts of the body, especially the skin, joints, blood, and kidneys. The body's immune system normally makes proteins called antibodies to protect the body against viruses, bacteria, and other foreign materials. These foreign materials are called antigens. In an autoimmune disorder such as lupus, the immune system loses its ability to tell the difference between foreign substances (antigens) and its own cells and tissues. The immune system then makes antibodies directed against "self." These antibodies, called "auto-antibodies," react with the "self" antigens to form immune complexes. The immune complexes build up in the tissues and can cause inflammation, injury to tissues, and pain. The disease whose severity spans a spectrum, with some lucky patients having milder symptoms without organ involvement (such as malar rash, canker sores, joint pain without swelling) and other patients having severe organ involvement (such as found in the kidneys and brain). Physicians who treat SLE will often describe the disease as mild, moderate or severe, usually on the basis of the severity of the organ involvement and the potential for permanent organ damage. Generally, no two people with systemic lupus will have identical symptoms. Systemic lupus may include periods in which few, if any, symptoms are evident ("remission") and other times when the disease becomes more active ("flare"). Most often when people mention "lupus," they are referring to the systemic form of the disease; however, there are two other types of lupus: discoid and drug-induced. Discoid (cutaneous) lupus is always limited to the skin. It is identified by a rash that may appear on the face, neck, and scalp. Drug-induced lupus occurs after the use of certain prescribed drugs. The symptoms of drug-induced lupus are similar to those of systemic lupus. The drugs most commonly connected with drug-induced lupus are hydralazine (used to treat high blood pressure or hypertension) and procainamide (used to treat irregular heart rhythms). The symptoms usually fade when the medications are discontinued. For most people, lupus is a mild disease but, it may cause serious and even life-threatening problems. More than 16,000 Americans develop lupus each year. It is estimated that 1.5 million Americans have been diagnosed with lupus. Women develop the disease at least ten times more often; although men and even children are affected. Africans Americans have a three-fold higher frequency of lupus than do European Americans, and lupus in African Americans is, on average, more severe. Genes causing lupus in African Americans generally different from those causing lupus in European Americans.

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Lupus Diagnosis

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Some physicians make a diagnosis on the basis of the 11 ACR classification criteria (see below). The criteria, however, were established mainly for use in scientific research (i.e., inclusion in randomized controlled trials), and patients may have lupus but never meet the full criteria. Click for Table 1982 Revised ARA Criteria for Classification of Systemic Lupus Erythematosus

ACR classification criteria

1. Serositis: Pleuritis (inflammation of the membrane around the lungs) or pericarditis (inflammation of the membrane around the heart);
2. Oral ulcers includes oral or nasopharyngeal ulcers.
3. Arthritis: nonerosive arthritis of two or more peripheral joints, with tenderness, swelling, or effusion;
4. Photosensitivity (exposure to ultraviolet light causes rash);
5. Blood-hematologic disorder-hemolytic anemia (low red blood cell count) or leukopenia ((low white blood cell count); Also lymphopenia, thrombocytopenia or hypocomplementemia;
6. Renal disorder: protein in urine or cellular casts
seen in urine under a microscope;
7. Antinuclearantibody test positive;
8. Immunologic disorder: Positive anti-Smith, anti-ds DNA, antiphospholipid antibody, and/or false
positive serological test for syphilis; Presence of anti-ss DNA in 70% of patients (though also positive in patients with rheumatic disease and healthy persons.
9. Neurologic disorder: Seizures or psychosis;
10. Malar rash (rash on cheeks);
11. Discoid rash (red, scaly patches on skin that cause scarring);

Discoid Rash

Malar "butterfly" Rash

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THE LUPUS DIAGNOSTIC TESTS: ANA: Antinuclear antibodies and other "Anti-"

The patient with SLE, or lupus is often first referred to a rheumatologist for diagnosis based on the result of a positive blood test for antinuclear antibodies (ANA). The ANA is the "sine qua non" of lupus, meaning that virtually every patient with SLE will have a positive ANA, yet the blood test is by no means "specific". This means that some 5% of the population may have a positive ANA but never develop lupus. In general, the higher the antibody titer value, the high the probability of SLE. A more specific blood test is the "anti-double stranded, or native, DNA". Finding this antibody in the patient's very genetic material makes the diagnoses of SLE more probable, and its presence may predict current or future involvement of the kidney. Perhaps the most specific antibody is "anti-Smith", names after the patient in whom it was originally found. This test is nearly 100% predictive of SLE if it is present, but it is only found in less than 20% of patients.

ANA test: measured in titers

An antinuclear antibody (ANA) test measures the amount and pattern of antibodies in your blood that work against your own body (autoimmune). The results of an ANA test are usually given in titers. A titer is a measure of how much the blood sample can be diluted before the presence of the antibodies can no longer be detected. [ Basically the titer its a ratio referring to a concentration of antibodies ] For example, a titer of 1 to 40 (1:40) means that antibodies can be detected when 1 part of the blood sample is diluted by up to 40 parts of a salt solution (saline). A larger second number means there are more antibodies in the blood. Therefore, a titer of 1 to 80 indicates more antibodies in the blood than a titer of 1 to 40. There are different subtypes of ANA which may have a range of normal values. Normal values may vary from lab to lab. Results will usually be available in about 1 week. Antinuclear antibodies, may have Normal titer of 1:40 or less.

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What does the ANA test result mean?

True Positive ANA

A positive ANA test result may suggest an autoimmune disease, but further specific testing is required to assist in making a final diagnosis. ANA test results can be positive in people without any known autoimmune disease. While this is not common, the frequency of a false positive ANA result increases as people get older.

Also, ANA may become positive before signs and symptoms of an autoimmune disease develop, so it may take time to tell the meaning of a positive ANA in a person who does not have symptoms. Most positive ANA results don't have significance, so physicians should reassure their patients but should also still be vigilant for development of signs and symptoms that might suggest an autoimmune disease.

About 95% of SLE patients have a positive ANA test result. If a patient also has symptoms of SLE, such as arthritis, a rash, and autoimmune thrombocytopenia, then he probably has SLE. In cases such as these, a positive ANA result can be useful to support SLE diagnosis. Two subset tests for specific types of autoantibodies, such as anti-dsDNA and anti-SM, may be ordered to help confirm that the condition is SLE.

A positive ANA can also mean that the patient has drug-induced lupus. This condition is associated with the development of autoantibodies to histones, which are water soluble proteins rich in the amino acids lysine and arginine. An anti-histone test may be ordered to support the diagnosis of drug-induced lupus.

A positive result on the ANA also may show up in patients with Sjögren’s syndrome, Scleroderma, Raynaud’s disease, rheumatoid arthritis, dermatomyositis, mixed connective tissue disease, and other autoimmune conditions.

A doctor must rely on test results, clinical symptoms, and the patient’s history for diagnosis. Because symptoms may come and go, it may take months or years to show a pattern that might suggest SLE or any of the other autoimmune diseases.

Negative ANA

A negative ANA result makes SLE an unlikely diagnosis. It usually is not necessary to immediately repeat a negative ANA test; however, due to the episodic nature of autoimmune diseases, it may be worthwhile to repeat the ANA test at a future date.

Aside from rare cases, further autoantibody (subset) testing is not necessary if a patient has a negative ANA result.

False positive results

The ANA test is said to be false positive when a person tests positive but does not have any other features of autoimmune disease . This situation occurs more often in women and elderly people. Certain medications, such as hydralazine, isoniazid, procainamide, and some anticonvulsant medications increase the chances of having a positive ANA test

Continue Reading: LabTestOnline.org & Uptodate.com

Advanced Reading:Five basic patterns are recognized in indirect immunofluorescence

FEATURED LUPUS DRUGS : CORTICOSTEROIDS: Prednisone and others

Corticosteroids, also called steroids, are a mainstay in the fight against lupus. Corticosteroids are actually hormonal drugs that behave like the body’s own adrenal gland hormone adrenaline. These synthetic versions are very powerful and the doses given are much greater than the body can produce on its own. Don't confuse corticosteroids with the anabolic steroids often abused by athletes. Corticosteroids affect lupus because they suppress the overactive immune system and inflammation contributing to the flares that may cause tissue damage. Unfortunately, these strong drugs have side effects and consequences that all lupus patients must discuss with their physicians before starting therapy. An immunosuppressed patient often suffers infections, such as strep throat, UTIs, or even a reactivated infection like herpes (shingles) or TB. There are many side effects of corticosteroids including high blood pressure, calcium loss, GI irritation and ulcers (thrush/ yeast infections), delayed wound healing, menstrual irregularities, increased appetite and weight gain, hair growth, especially on arms, face and legs, fat deposits on face and upper body (Also called “moonface” and “buffalo hump”). Long term use and high doses can lead to some secondary health conditions such as hypertension, osteoporosis, exogenic Cushing's syndrome, cataracts, glaucoma, and even diabetes. While high doses or long-term therapy increase the risk of side effects, stopping therapy too abruptly carries its own dangers. I can’t stress this enough: NEVER stop taking any form of corticosteroids without discussing it with your physician first! While you are taking corticosteroids, your body’s adrenal gland, realizing that there are plenty of hormones around, stops making the usual amounts of cortisone that it usually does. If you stop taking those steroids suddenly, the adrenal gland won’t have time to immediately start up and make enough to keep your body functioning and you can suffer acute adrenal insufficiency, a serious and potentially fatal development. If the steroids aren’t weaned off slowly over a period of time, you run the risk of lupus flares or worsening symptoms. Additionally, if a person on long-term steroid therapy is going to undergo surgery or childbirth, or has any sort of trauma, they may need additional doses to compensate for the stress to the body.

Prednisone Tablets

Steroids come in varying forms, depending on the reason and strength that they are needed. Prednisone is an oral pill that comes in varying strengths from 1 mg to 50mg. You can take them once or twice a day, or every other day, depending on your symptoms and prescription. This is probably the most common form of steroid therapy for lupies because we can administer it ourselves at home, without the need for a nurse or doctor. Topical corticosteroids are commonly used on lupus rashes. It’s available as a cream, ointment or lotion, depending on the area needing treatment. While you can avoid some of the systemic side effects that come with other types of steroids, topical meds can carry other risks you should know about, like hypopigmentation (lightening of the skin), burning, irritation or dryness. Check with your physician to see if this type of medication might help you lower or eliminate the need for oral steroids. Depo-Medrol is an intramuscular injection, given in the gluteus or the deltoid. (Backside or arm) It lasts longer than an equivalent dose of oral medication, but has to be administered by a healthcare professional, which means either home health care or a doctor’s office visit. There is also an intravenous variation of Depo-Medrol. Also known as Solu-Medrol, it works immediately, and can last a week. Because of its fast onset of action, it’s the preferred method of administering steroids to people with adrenal insufficiency. However, because of the need for IV access, at least an outpatient hospital admission is usually necessary. (Unless you have a portacath or other permanent IV access, in which case it can be given by a nurse at home.)

It’s important for anyone undergoing corticosteroid therapy to discuss any questions or concerns with her physician both before and during the therapy. As tempting as it might be for the experienced lupie to "juggle" her own doses, it’s a very risky proposition, and it’s better to talk honestly with your docs in order to avoid a dangerous situation. Remember that you’re always better off knowing more about your available treatments, and that knowledge is power. Get the information that you need to be an informed patient advocate for yourself!

FEATURED SECONDARY CONDITION: OSTEOPOROSIS

Osteoporosis occurs when the body fails to form enough new bone, or when too much old bone is reabsorbed by the body, or both. Thin weakened bones are more likely to fracture. Fractures of the vertebrae, wrists, or hips are usually the first indication. Symptoms such as lower back, neck, bone pain or tenderness may not occur until late in the disease. Loss of estrogen in older women is the most common cause associated with this condition. Corticosteroids ( Prednisone for example) can lead to a loss in bone density and may cause "secondary" osteoporosis in men and women of any age. Postmenopausal women taking corticosteroids are at a very great risk. Calcium and phosphate are two minerals that are essential for normal bone formation. Throughout youth, the body uses these minerals to produce bones. If calcium intake is not sufficient, or if the body does not absorb enough calcium from the diet, bone production and bone tissues may suffer. Steroids { corticosteroids) inhibit the body's absorption of calcium through the intestine then encourage calcium loss via the kidneys. A very serious condition can result from damaged bone cells deprived of blood supply by fatty clots from steroids. The lack of of oxygen leads to "dead bone" called avascular necrosis. Counteracting calcium depletion, slowing down or stopping bone loss, preventing bone fractures by minimizing the risk of falls, and controlling pain associated with the disease are the obvious goals for combating this health condition.

TESTS: Bone mineral density (BMD) or DEXA scans

Bone mineral density (BMD) testing -- as performed in dual-energy x-ray absorptiometry (DEXA) -- measures the demineralization of the bones. This has become the gold standard for evaluation for osteoporosis. The cost for DEXA scans run $200 - $300; but most insurance plans cover them. Anyone taking prednisone ( corticosteroids ) should be tested at least every one or two years; however, early on a patient should have a baseline bone-density test due to the rapid bone loss that takes place the first two years of steroid use.

OSTEOPOROSIS DRUGS: Actonel, Fosamax, Evista, et al

There are several different kinds of drugs used to treat osteoporosis. They vary in their side effects, benefits, and costs. Because of potential harm to a developing fetus, it is extremely important that women not only communicate to their doctors that they are pregnant but whether they have any plans to become pregnant. Lingering drugs may still affect pregnancy, so treatment and prevention may have to be through diet and exercise.

Convenient Packaging for Monthly Supply of Actonel

Bisphosphonates are a type of drug approved for both the prevention and treatment of osteoporosis. The two most well known marketed drugs -- alendronate (Fosamax) and risedronate (Actonel) -- prevent existing bone loss and reduce the risk of spinal and hip fractures.

While side effects are generally mild, potential side effects include stomach upset and irritation of the esophagus. Because bisphosphonates are difficult to absorb, these medicines should be taken on an empty stomach. The patient should not lie down or consume food or beverages (other than water) for at least 30 minutes after taking the medicine. The physician may also recommend taking calcium and Vitamin D supplements. If these drugs are not successful patients may have other options.

Raloxifene (Evista) is another drug used for the prevention and treatment of osteoporosis. Raloxifene is similar to the breast cancer drug tamoxifen. Raloxifene can reduce the risk of spinal fractures by almost 50%. (It does not appear to prevent other fractures, including those in the hip.) It may have protective effects against heart disease and breast cancer, though more studies are required. The most serious side effect of raloxifene is a very small risk of blood clots in the leg veins (deep venous thrombosis) or in the lungs (pulmonary embolus).

Patients may be candidates for synthetic parathyroid hormone -- teriparatide (Forteo) -- a self administered daily injection that stimulates new bone and significantly increases bone mineral density. Side effects may include nausea, leg cramps or dizziness and should not be used before menopause or by those who have undergone radiation therapy. Another option -- calcitonin (Miacalcin) -- available as an injection or nasal spray. It slows bone loss and increases bone density of the spine in women that are post menopausal by more than 5 years. The injections may result in allergic reactions, and the nasal spray may cause bloody nose. Other possible side effects are similar to the bisphosphonates.

On the horizon a new drug, Prestara, a mild androgenic hormone, may be approved for the future and may even be safe for women who desire to get pregnant.