Flares and What Triggers Lupus

In the simplest of terms, a lupus flare is when a person's lupus has been quiet or in remission, and then it becomes active again, either with new symptoms or a recurrence of old ones.

So what triggers an attack of lupus in someone? Scientists have noted some common features in many lupus patients. In some, exposure to the sun causes the sudden development of a rash, and then possibly other symptoms. In others, an infection ... perhaps a cold or a more serious infection ... does not get better, and then complications arise. These complications may be the first signs of lupus.

In still other cases, a drug taken for some illness produces the signaling symptoms. In some women, the first symptoms and signs develop during pregnancy. In others, they appear soon after delivery. Many people cannot remember or identify any specific factor. Obviously, many seemingly unrelated factors can trigger the onset of the disease.

Keeping a Flare at Bay

- Always take your medication as prescribed.
- Keep your doctor's appointments, even if you're feeling well.
- Avoid taking sulfa drugs (sulfonomides) that are used to treat infections such as bronchitis and urinary tract infections.
- Limit your exposure to sunlight; apply sunscreen before going outside during the day, and - ---- wear sun-protective clothing and a wide-brimmed hat.
- Reduce your risk of infections.
- Get plenty of rest.
Do not smoke.

The Causes of Lupus

The cause(s) of lupus is currently unknown, but there are environmental and genetic factors involved. Some environmental factors which may trigger the disease include infections, antibiotics (especially those in the sulfa and penicillin groups), ultraviolet light, extreme stress, certain drugs, and hormones.

Scientists believe there is a genetic predisposition to the disease, as lupus is known to occur within families. However, there is no known gene or genes which are thought to cause the illness. There are recent discoveries of a gene on chromosome 1 which is associated with lupus in certain families. Previously, genes on chromosome 6 called "immune response genes" were also associated with the disease. Only 10 percent of lupus patients will have a close relative (parent or sibling) who already has or may develop lupus. Statistics show that only about five percent of the children born to individuals with lupus will develop the illness.

Lupus is often called a "woman's disease" despite the fact that many men are affected. Lupus can occur at any age, and in either sex, although it occurs 10-15 times more frequently among adult females than among adult males after puberty or after the emergence into sexual maturity. The symptoms of the disease are the same in men and women. People of African, American Indian, and Asian origin are thought to develop the disease more frequently than Caucasian women. The reasons for this ethnic selection are not clear.

Hormonal factors may explain why lupus occurs more frequently in females than in males. The increase of disease symptoms before menstrual periods and/or during pregnancy support the belief that hormones, particularly estrogen, may somewhat regulate the way the disease progresses. However, the exact reason for the greater prevalence of lupus in women, and the cyclic increase in symptoms, is unknown.

Lupus Lab Tests: Other Autoantibodies

In those individuals with a positive ANA, additional tests can be done for certain particular antibodies that may better establish a diagnosis of SLE. The knowledge of which particular antibody is responsible for the positive ANA test can sometimes be helpful in determining which autoimmune disease is present. For instance, antibodies to DNA (the protein that makes up the body's genetic code) are found primarily in SLE. Antibodies to histones (DNA packaging proteins) are usually found in people with drug-induced lupus, but may also be found in those with SLE. Antibodies to the Sm antigen are found almost exclusively in lupus, and often help to confirm the diagnosis of SLE. Antibodies to RNP (ribonucleoprotein) are found in a number of connective tissue diseases. When present in very high levels, RNP antibodies are suggestive of mixed connective tissue disease, a condition with symptoms like those of SLE, polymyositis, and scleroderma.

Antibodies to Ro/SS-A are found in people with either lupus or Sjogren's syndrome, and are almost always found in babies who are born with neonatal lupus. Antibodies to Jo-1 are associated with polymyositis, while antibodies to PM-Scl are associated with certain cases of polymyositis that also have features of scleroderma. Antibodies to Scl-70 are found in people with a generalized form of scleroderma, and antibodies to the centromere (a structure involved in cell division) are found in people with a limited form of scleroderma which tends to have a chronic course.

Complement
Laboratory tests which measure complement levels in the blood may also be helpful to the physician in making a diagnosis of SLE. Complement is a blood protein that destroys bacteria and also influences inflammation. Complement proteins are identified by the letter "C" and a number. The most common complement tests are C3, C4, and CH50. If the total blood complement level is low, or the C3 or C4 complement values are low and the person also has a positive ANA, some weight is added to the diagnosis of lupus. Low C3 and C4 complement levels in individuals with a positive ANA may signify the presence of active disease, especially kidney disease.

Biopsy

Sometimes examination of a tissue sample (biopsy) can be helpful in making a diagnosis. The biopsy is one of the best ways to evaluate an organ or tissue. The procedure involves removal of a small sliver of tissue, which is then examined under a microscope. The doctor can use the biopsy to identify the amount of inflammation and damage to the tissue. Further tests can be performed on the specimen to determine whether the problem is due to lupus or is caused by some other factor such as infection or medication. Almost any tissue can be biopsied. The most common sites biopsied in lupus are the skin and kidney. The results of the biopsy, like any other laboratory test, should be examined in combination with the individual's medical history and clinical findings.

Tests to Assess Disease Activity

When a person diagnosed with lupus develops new or recurring symptoms, laboratory testing of blood or urine can help determine if the symptoms are due to an increase in lupus activity. Disease activity correlates with a rise in:

-CRP (C-reactive protein) binding
-Sedimentation rate, or ESR
-Anti-DNA
-Liver and kidney function tests (AST, ALT, BUN, creatinine)
-CPK (muscle enzyme)
-Urine protein or cellular casts

Disease activity also correlates with a fall in:

-CBC (white blood cell count, hemoglobin, platelets)
-Complement components
-Serum albumin

Putting It All Together

The interpretation of all these tests, and their relationship to symptoms, can be difficult. When a person has many symptoms and signs of lupus and has positive tests for lupus, it is easier for physicians to make a correct diagnosis and begin treatment. It is more common for an individual to report vague, seemingly unrelated symptoms of achy joints, fever, fatigue, or pain, and to have negative or borderline test results. Fortunately, with growing awareness of SLE, an increasing number of physicians will consider the possibility of lupus in the diagnosis. While these tests are useful only when their strengths and limitations are understood, in the hands of skilled physicians these are important tools that assist in diagnosing lupus.

Lab Tests for Lupus: The Antinuclear Antibody (ANA or FANA) Test

The immunofluorescent antinuclear antibody (ANA or FANA) test is a sensitive test for lupus, since it is present in 97 percent of those with the disease. When three or more typical clinical features are present, such as skin, joint, kidney, pleural, pericardial, hematological, or central nervous system findings as described above, a positive test confirms the diagnosis.

The ANA test is positive in almost all individuals with systemic lupus, and is the most sensitive diagnostic test currently available for confirming the diagnosis of systemic lupus when accompanied by typical clinical findings. A negative ANA test is strong evidence against lupus as the cause of a person's illness, although there are very infrequent instances where SLE is present without detectable anti-nuclear antibodies. ANA-negative lupus can be found in people who have anti-Ro (SSA) or antiphospholipid antibodies. However, a positive ANA test, by itself, is not proof of lupus since the test may also be positive in:

• other connective tissue diseases such as scleroderma, Sjogren’s syndrome, rheumatoid arthritis, and thyroid disease, as well as liver disease and juvenile arthritis
• individuals being treated with certain drugs, including procainamide, hydralazine, isoniazid, and chlorpromazine
• viral illnesses such as infectious mononucleosis, and other chronic infectious diseases such as hepatitis, lepromatous leprosy, subacute bacterial endocarditis, and malaria
• other autoimmune diseases, including thyroiditis and multiple sclerosis

The test can even be weakly positive in about 20 percent of healthy individuals. While a few of these healthy people may eventually develop lupus symptoms, the majority will never develop any signs of lupus or related conditions. The chances of a person having a positive ANA test increases as he or she ages.

Finally, as many as 30-40 percent of asymptomatic first degree relatives (siblings, parents, and children) of people with lupus may have a positive ANA test.

ANA Titers (number) and Patterns

ANA reports include a titer (pronounced TY-tur), and a pattern. The titer indicates how many times the lab technician had to dilute plasma from the blood to get a sample free of the antinuclear antibodies. For example, a titer of 1:640 shows a greater concentration of anti-nuclear antibodies than a titer of 1:320 or 1:160.

The apparent great difference between various titers can be misleading. Since each dilution involves doubling the amount of test fluid, it is not surprising that titers increase rather rapidly. In actuality, the difference between a 1:160 titer and a 1:320 titer is only a single dilution. This does not necessarily represent a major difference in disease activity. ANA titers go up and down during the course of the disease, and a high or low titer does not necessarily mean the disease is more or less active. Therefore, it is not always possible to determine the activity of the disease from the ANA titer.

A titer above 1:80 is usually considered positive. However, some laboratories may interpret different titer levels as positive, so one cannot compare titers from different laboratories.

The pattern of the ANA test can sometimes be helpful in determining which autoimmune disease is present and which treatment program is appropriate. The homogeneous (smooth) pattern is found in a variety of connective tissue diseases, as well as in people taking particular drugs such as certain anti-arrhythmics, anti-convulsants or anti-hypertensives. This pattern is also the one most commonly seen in healthy individuals who have positive ANA tests. The speckled pattern is found in SLE and other connective tissue diseases, while the peripheral (rim) pattern is found almost exclusively in SLE. The nucleolar (a pattern with a few large spots) pattern is found primarily in people who have scleroderma.

Because the ANA is positive in so many conditions, the results of the ANA test have to be interpreted in light of the person's medical history, as well as his or her clinical symptoms. Thus, a positive ANA alone is never enough to diagnose lupus. On the other hand, a negative ANA argues against lupus but does not rule out the disease completely.

A Positive ANA Does Not Equate to Having a Disease

The ANA should be looked at as a screening test. If it is positive in a person who is not feeling well and who has other symptoms or signs of lupus, the physician will probably want to conduct further tests for lupus. If the ANA is positive in a person who is feeling well and in whom there are no other signs of lupus, it can be ignored. If there is any doubt, a consultation with a rheumatologist should clarify the situation.

Coping with Lupus

Learning to live with lupus is like anything else in life; like becoming a parent, starting a new job, making an investment or buying a car. One has to learn how to do it and this learning does not happen overnight. Life is made up of pleasures and of obligations. Clearly, lupus can be quite an obligation but, depending on the person, the burden of this obligation may be lighter or heavier.

One key to coping with the illness is to get organized. Managing the day to day aspects of your lupus will help you to be in better control. Below I have listed some practical tips that will help you take care of your lupus responsibilities.

1.See your doctor regularly and do not postpone your visit because you "feel so well". Make a list of all your questions and bring it to your next appointment.

2.Make a list of all your pills or medications to each appointment. This helps your doctor identify "that little yellow pill" that was prescribed by "that other physician". Knowing all your medications, your doctor may be able to cut down on the number you are taking, which is nice and saves money too.

3.Buy a multi-compartment pill box in any pharmacy so that you can lay out your pills for each day or week. This makes it easy to check if you have forgotten to take your medications.

4.If your doctor orders a urine collection over 24 hours, collect your urine from Sunday to Monday morning and bring it into the hospital on your way to work. Carrying around a jug of urine may be embarassing but do your best not to lose a drop. Important decisions about the management of your disease will be made, in part, based on the results of this collection. (Urine collection should not be done during menstruation.)

5.If your blood pressure is checked by your family doctor, the nurse at work or by yourself, don't forget to bring these readings to your next appointment. (Readings done at the Lupus Clinic are often higher and this can cause unnecessary concern.)

6.Purchase a thermometer. If you feel that you are running a fever, take your temperature every six hours and wrrite down the results. If the problem continues and is not explained by a cold or the flu, call your physician.

7.Be prepared for blood tests to be taken regularly, even when everything seems fine. SLE is unpredictable and the sooner a flare is detected, the better.

Lab Tests for Lupus

Because many symptoms of systemic lupus erythematosus (SLE) mimic those of other illnesses, lupus can be a difficult disease to diagnose. Diagnosis is usually made by a careful review of three factors:

1. the individual's entire medical history;
2. the individual's current symptoms; and
3. an analysis of the results obtained in routine laboratory tests and some specialized tests related to immune status.

To make a diagnosis of SLE, an individual must show clinical evidence of a multi-system disease (i.e. has shown abnormalities in several different organ systems). The following are typical symptoms or signs that might lead to suspicion of SLE:


1. Skin: Butterfly rash across the cheeks; ulcers in the mouth; hair loss.
2. Joints: Pain; redness, swelling.
3. Kidney: Abnormal urinalysis suggesting kidney disease.
4. Lining membranes: Pleurisy (inflammation of the lining of the lung); pericarditis (inflammation of the heart lining); and/or peritonitis (inflammation around the abdomen). Taken together, these types of inflammation are known as polyserositis.
5. Blood: Hemolytic anemia (the red cells are destroyed by autoantibodies); leukopenia (low white blood cell count); thrombocytopenia (low number of platelets).
Lungs: Infiltrates (shadowy areas seen on a chest x-ray) that come and go
6. Nervous system: Convulsions (seizures); psychosis; nerve abnormalities that cause strange sensations or alter muscular control or strength.

If an individual has several of these symptoms, the physician will then usually order a series of tests to examine the functioning of the individual's immune system. In general, physicians look for evidence of autoantibodies. Although there is no one test that can definitely say whether or not a person has lupus, there are many laboratory tests which aid the physician in making a lupus diagnosis.

First, there are routine clinical tests which suggest that the person has an active systemic disease. These include the sedimentation rate (ESR) and CRP which are frequently elevated in inflammation from any cause. Serum protein electrophoresis may reveal increased gammaglobulin and decreased albumin, and routine blood counts may reveal anemia and low platelet and white cell counts. Finally, routine chemistry panels may reveal kidney involvement by increases in serum blood urea nitrogen and creatinine, abnormalities of liver function tests, and increased muscle enzymes (such as CPK) if muscle involvement is present. These kinds of abnormalities alert the doctor to the presence of a systemic disease with multiple organ involvement.

Commonly used blood tests in the diagnosis of SLE are:

1. The anti-nuclear antibody test (ANA) to determine if autoantibodies to cell nuclei are present in the blood
2. The anti-DNA antibody test to determine if there are antibodies to the genetic material in the cell
3. The anti-Sm antibody test to determine if there are antibodies to Sm, which is a ribonucleoprotein found in the cell nucleus
4.Tests to examine the total level of serum (blood) complement (a group of proteins which can be consumed in immune reactions), and specific levels of complement proteins C3 and C4

In coming posts I will be goin through these Blood tests individualy..

Symptoms of SLE Continued

3. Discoid lupus is a scaly, raised rash that appears on the face, scalp, ears, chest or arms in 25% of SLE patients. The rash, if untreated, may leave permanent scars. Involvement of the hair roots on the scalp may also cause permanent damage in the form of bald spots but widespread or total baldness is rare. The rash is photosensitive (it will get worse after being in the sun) but this can be avoided if the proper sunscreens are used. In Canada, patients typically experience the reappearance or worsening of discoid lesions during spring and summer. The use of a sunscreen for protection and appropriate treatments can usually reduce or control this problem. It is important to be aware that approximately 10% of discoid lupus patients will eventually develop a usually mild form of SLE. As a result, discoid lupus patients are tested for SLE from time to time.



4. Mucosal ulcers are small sores that occur on the mucous lining of the mouth or nose in as many as 40% of SLE patients. Mouth ulcers most often occur on the roof of the mouth and, because these are usually painless, the patient may not notice them except, perhaps, when eating spicy foods. Painful canker sores happen to lupus patients too but are different from lupus ulcers. Nose ulcers are usually painless but they may cause nosebleeds which, if frequent, should be brought to the physician's attention.



5. Arthritis is very common in SLE. In fact, it is unusual for patients with SLE not to have arthritis sooner or later. It causes pain in the joints of the hands, wrists, elbows, knees or other joints in the arms and legs. (Lupus arthritis does not cause back pain although patients may experience back pain from other causes.) The pain may be migratory, meaning that it goes from one joint to another, and it may occur in the same joint on both sides of the body. Signs of inflammation (swelling, redness, heat) may not show up but, when SLE is very active, the inflammation and pain may be severe.Both the patient and the physician can become very frustrated by the arthritis of SLE. Because the pains move from joint to joint and can appear and disappear quite quickly, the patient may not have any signs of arthritis at the time of their appointment. As a result, diagnosing arthritis in the patient can take quite a long time.
Lupus arthritis is usually not crippling and patients should not fear becoming wheelchair bound (although the use of a wheelchair is sometimes necessary in SLE due to causes other than arthritis). Changes (deformities) in the fingers do sometimes happen as a result of arthritis but this does not usually cause a major loss of function.



6. Pleuritis is inflammation of the pleura, the lining of the lungs, and pericarditis is inflammation of the pericardium, the lining of the heart. These problems may occur alone or together and affect at least 50% of all SLE patients. Pleuritis, and sometimes pericarditis, cause a particular type of chest pain that gets worse when breathing in and gets better or goes away when breathing out. Patients may also have less pain when sitting or standing and more pain when lying down. If the pain is severe, the person may be limited to shallow breathing only. Lupus patients who experience a second bout of pleuritis or pericarditis later in the course of their disease often readily point out that "...the pain is like it was the first time...".
While there are many benign (not serious) causes of chest pain, it is wise to inform your doctor immediately if you experience such a pain. By examining you, and performing tests such as a chest x-ray, heart ultrasound and electrocardiogram (ECG), your physician will be able to make a diagnosis and, if necessary, start treatment.



7. Kidney involvement ranges from very mild to very serious. It is believed that most, if not all, patients with SLE have at least some mild degree of kidney (or renal) involvement. When mild, this involvement is usually not a problem. However, in 50% of patients, kidney damage may occur and this is of concern because it may lead to a loss of the kidney's ability to clean the blood. An upsetting feature of SLE kidney involvement is that it tends to remain silent (the patient will not have any symptoms) until damage has already occurred. Then the patient will complain of weight gain and swelling, particularly in the feet and legs. Doctors call this fluid retention or oedema and it is the most common symptom of major kidney involvement.
It is very important to detect kidney involvement early because some of the deaths caused by SLE are related to severe kidney involvement. For this reason, regular tests of kidney function (simple urine and blood tests) will be ordered by your physician. Urine analysis and tests of kidney function are best performed from time to time, even when lupus seems to be quiet. Regular testing of the kidneys is absolutely necessary whenever lupus is active. Urine analysis can show various signs of inflammation in the kidney such as clumps of red blood cells (called cylinders or casts) or the presence of an excessive amount of protein. To determine how much involvement is present, doctors measure the amount of protein and the performance of the kidneys on all your urine collected over a 24 hour period. In this way, early involvement of the kidneys can be discovered and, if necessary, treatment can be started.Although most patients with SLE never develop kidney involvement that requires the use of artificial blood filtration (dialysis), some patients clearly will. Because kidney involvement can be so serious, affected patients should be evaluated by a nephrologist, a physician who specializes in treating diseases of the kidney.



8. Seizures (epilepsy) and psychosis are serious problems caused by central nervous system (CNS) involvement in SLE, and occur in 15-25% of patients. These problems can be caused by many disorders other than SLE so doctors must take the time to rule out other conditions before deciding that SLE is the true culprit. It is important to be aware that, with CNS involvement, other types of problems may occur which are not as serious as seizures or psychosis.Seizures most commonly involve loss of consciousness and involuntary body movements. The person does not usually recall what happened and descriptions by relatives or friends who witnessed the seizure are most helpful. Seizures can usually be confirmed by performing an electroencephalogram (EEG) which is a reading of the electrical activity of the brain. Fortunately, several drugs are available to control seizures in SLE patients.When seizures are the first symptom experienced by the patient, SLE may not be suspected until other lupus problems appear. This can cause confusion, as physicians may wonder if the other symptoms were triggered by the anti-epileptic drugs. In other words, is this drug-induced lupus? In most cases the answer is no and the seizures will begin again if the anti-epileptic drug is stopped.Psychosis is a serious mental condition where thinking and behavior are disturbed, often including hallucinations (seeing or hearing things that aren't there) and delusions (false notions or ideas, for instance the belief that one is being poisoned). Relatives may say that the person has partly or completely lost contact with reality. Other symptoms may be present such as confusion (the person does not know where he/she is, what the time and date are and may not recognize family members or close friends). Many doctors think that SLE psychosis is best treated with prednisone or a steroid drug.



9. Disorders of the blood cells are caused by autoantibodies that attack one of the blood cells or particles, usually the red or white cells or platelets. It is important to understand that these problems may have causes other than SLE. In all cases, doctors must rule out the other possible causes before treating these problems as related to lupus. Red blood cells may be attacked, resulting in a large number of cells being destroyed and removed from the body in the spleen, a process called hemolytic anemia. This destruction may be slow and relatively mild or may be very quick and cause an emergency. Prednisone is effective in treating this anemia in most people but sometimes the spleen must be removed to bring the anemia under control.
SLE commonly causes a decrease in the white blood cells called leukopenia. In most instances this is not dangerous and by itself does not require treatment.Platelets are cell particles that control the clotting of blood. Autoantibodies to platelets may cause the platelet count to drop, a situation called thrombocytopenia. Some SLE patients continue to have, over time, lower than normal platelet counts which do not require treatment but do need to be checked regularly. In others, a severe decrease in the platelet count can cause bleeding in various parts of the body such as the digestive tract, the urinary tract, the uterus or the brain. This is a serious situation that requires treatment with prednisone and, sometimes, removal of the spleen. Easy bruising of the skin may be a sign of a decreased platelet count but it also occurs in normal individuals or can be caused by prednisone. In my experience, these explanations account for far more cases of bruising than does a low platelet count.



10. Immunologic disorders refer to four autoantibodies found in the blood which, when taken with other symptoms, point to SLE.The lupus erythematosus cell preparation (doctors say LE prep) test is positive when a particular cell (the LE cell) is found in the blood of patients with active SLE. However, the LE cell is sometimes found in disorders other than SLE and most doctors have stopped using this test simply because better tests are now available.Anti-native DNA autoantibodies are common in SLE and it would be unlikely that they would be seen in other diseases. This test is repeated often because the amount of anti-native DNA autoantibodies seems to increase when lupus is active and the test can help the physician measure the degree of disease activity.Anti-Sm autoantibodies refer to the name of the first patient in whose blood they were found (her name was Smith). These antibodies point to SLE.A false-positive test for syphilis, which happens in approximately 20% of SLE patients, suggests that patients have the venereal disease called syphilis when, in fact, they don't. This is another of SLE's sneaky thousand faces and one that has caused a lot of fright and upset. When a diagnosis of SLE is suspected, doctors simply request this blood test for syphilis (V.D.R.L.) and, if it is positive, further tests are done that can show whether or not syphilis is actually present.



11. Antinuclear antibodies, or ANA, are found in the blood in almost every patient with SLE. This test has made the diagnosis of SLE more certain, as it is very accurate, but three cautions must be kept in mind. The first is that ANA may be present as a reaction to various drugs, may be present in diseases other than SLE and may be present in healthy individuals, particularly the elderly. Therefore, a positive ANA must always be looked at in light of other symptoms of SLE. The second caution is that the improved methods of testing for ANA have found small amounts of ANA in the blood of many apparently healthy people. Therefore a positive ANA does not necessarily mean a diagnosis of SLE. The third caution is that a positive ANA test should be repeated to be absolutely sure.In rare cases, a diagnosis of SLE will be made even when the ANA test is not positive. Some patients with several specific symptoms of lupus will not develop a positive ANA until later on in their disease. Other patients may have a particular autoantibody in their bloodstream called anti-Ro (from Robert who was the first person identified with this antibody) which is poorly detected by standard ANA tests. In these cases, there is a specific test for anti-Ro that is available at University Hospitals.