Living with Lupus what to expect

A chronic illness, lupus is different for each individual, but it often appears in cycles, which can consist of:

- a 'flare', with severe acute symptoms needing medical attention;

- a 'chronic' phase, when symptoms may continue but are less severe;

- a 'remission', when symptoms may disappear completely for long periods, but can return.

In the chronic phase, and especially in the remission phase (when it is easy to forget to take care), a person with lupus needs to avoid situations that can bring on a flare. These include getting too tired, intense stress, poor diet or other factors noticed by patient or doctor.

Lupus is a serious condition, but diagnosis and treatment are improving. Today it can be treated and controlled. More and more people with lupus are finding that they can be active and productive.

S.L.E Foundation : Life without Lupus Video

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.