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LUPUS: The Disease with a Thousand FacesTreating lupus with medications
The vast majority of SLE patients can be treated successfully. Some patients have mild SLE that does not require any treatment although regular follow-up is still necessary. Even SLE patients who have required treatment can reasonably hope that a remission will occur when little or no treatment will be needed.
Ideally there should be only one physician in charge of supervising your illness and treatment and this approach is generally followed in the University Hospital Lupus Clinics across Canada. In these clinics, most patients are treated by physicians who have specialized in rheumatology (called rheumatologists). These physicians have received a particularly specialized training in lupus. As needed, consultation with other specialists is sought and many patients are also followed by physicians specialized in skin care (dermatologists), blood diseases (hematologists), kidney diseases (nephrologists) or immunologic diseases (immunologists).
In dealing with lupus, two approaches are used. First, the symptoms are treated if necessary and secondly, people with SLE are advised to avoid certain things that are known to cause a flare in some patients. In this chapter, I will discuss the types of medications used to treat the symptoms of SLE.Drugs for arthritis
Arthritis is probably the most common symptom of SLE and when it is mild no treatment may be needed. If the pain is more severe, many patients are satisfied with a basic pain medication such as acetaminophen (for instance, Tylenol or Atasol). Non-steroidal anti-inflammatory drugs (called NSAIDS for short) are often used when acetaminophen doesn't control the pain of arthritis. There are over 20 different drugs in this family of medications, including aspirin. Although some of the NSAIDS are available over the counter, it is best left to your doctor to decide which one you should use. Not every drug works well for every patient and you may have to try several NSAIDS before finding one that relieves your pain.
The NSAIDS may cause many side-effects, from stomach upset to changes in kidney function. Several of these side-effects can cause trouble as they may imitate lupus problems or complicate lupus problems that already exist. For this reason, NSAIDS are often prescribed to be taken for a short time only, with instructions to decrease the dose as the arthritis improves.Hydroxychloroquine and chloroquine
The drugs hydroxychloroquine (Plaquenil ®)and chloroquine (Aralen ®) are very useful in treating sun sensitive skin rashes such as the discoid or the subacute types of lupus rashes. In many cases, rheumatologists prefer to use hydroxychloroquine because it is considered to be less toxic. However, dermatologists may prefer to use chloroquine to treat lupus rashes. This drug may also be tried if hydroxychloroquine is ineffective. While a sunscreen may often provide sufficient protection, some patients do get skin rashes in spite of the proper use of a good sunscreen. In these patients, hydroxychloroquine may be started in the spring and continued until the late fall.
Hydroxychloroquine is also used to treat lupus fatigue, arthritis and other milder symptoms of SLE. A recent study in several Canadian University Hospital Lupus Clinics has shown that hydroxychloroquine can help prevent flares in patients who have taken this medication continuously over a six month period.
At the relatively low dosage used in SLE (the dose is calculated based on the patient's weight), hydroxychloroquine seems to cause few problems. Apart from the pill's bad taste, the most common unwanted effect is some stomach upset. However, if hydroxychloroquine and chloroquine are taken in a high dose and over a long period of time, they may accumulate in the background of the eye (retina) and cause a loss of vision. In rare cases, blindness may occur. In over ten years of supervising patients on hydroxychloroquine, I have never seen a single case of damage to the eye but my more senior colleagues tell me that I probably will, eventually.
A series of precautions are taken to make certain that damage to the eye does not occur. Dosage is based on the body weight and, if possible, hydroxychloroquine is only used from spring to fall when the sun is the strongest. (However, many patients must take hydroxychloroquine for much longer periods.) Before hydroxychloroquine or chloroquine is started, patients must have their eyes examined by an eye doctor (ophthalmologist) and this examination should be repeated every six months. As well, patients started on these drugs should receive an Amsler grid and be told how to use it. The Amsler grid is a sheet of paper with many small squares (like a piece of graphing paper). Patients should inspect the squares regularly and if the lines are not clear, straight and square, they should see their eye doctor immediately. In my experience with hydroxychloroquine, eye damage will not get worse and sight will not be threatened if the drug is stopped at this early stage.Cortisone and Prednisone
In my view, the single most important factor explaining the remarkable improvement in life expectancy of SLE patients has been the discovery of the steroid drug cortisone in 1948 and, shortly after, the related drug prednisone. Although the term cortisone continues to be used by patients and the public, SLE patients are now treated with the much more effective drug prednisone. This drug is the best inflammation-fighting drug available at the moment. Prednilosone is also used in Canada and, for some SLE patients, is the best drug available. These drugs are sometimes referred to as steroids, but they are not related to testosterone and the other anabolic steroids used by some athletes to build muscle.
The decision to prescribe prednisone is never made lightly by your physician. Prednisone is considered if the symptoms are not being well controlled by other treatment(s), if there is a concern that SLE may quickly get out of control unless prednisone is used or if SLE is severe and perhaps life-threatening.
On the bright side, prednisone is necessary for the successful treatment of many lupus patients. For some, it is a lifesaving drug and the single best drug available. However, this powerful drug has a dark side too. There is no doubt that prednisone can cause a wide variety of side-effects in both the short and the long term. If you are taking prednisone, it is important for you to know what these problems are. This information is included in Appendix Two at the back of the booklet, "Side-effects of prednisone". Patients will be happy to know that research is underway to find steroid drugs which would have the powerful inflammation-fighting action of prednisone with fewer unwanted side-effects.
At times, patients may be tempted to stop taking prednisone or to reduce or increase their dose. Perhaps they are fed up with the side-effects or perhaps they are feeling better or worse. It is important that patients taking cortisone understand how cortisone works and why stopping or changing the medication without the supervision of the physician is dangerous.
Cortisone is a chemical that is normally produced by the body, not a foreign substance. In fact, the body has a vital need for cortisone. When prednisone treatment is started, the body responds by stopping its own production of cortisone and the adrenal glands that produce it get sluggish and lazy. If prednisone is used for a period of time and then suddenly stopped, the body may not be able to start producing enough cortisone for some time. The result could be a life-threatening lack of cortisone (adrenal insufficiency). So prednisone should never be stopped suddenly. If you are tempted to stop taking your prednisone, do not do it. Rather, discuss the situation with your physician.
Other patients decrease or increase their dose on their own. This is unacceptable. If you feel well enough that you think prednisone could be decreased or if you feel worse and think that it should be increased, call your doctor. Never forget that both you and your doctor have worked very hard to improve your health. You should not put your improvement at risk by making any changes to your therapy.A short history of steroid use
The first medical report on the use of cortisone in severe SLE was in 1957 but the doses used were too low and the results were disappointing. In the early sixties, high doses of prednisone (meaning one milligram or more for each kilogram of body weight) were used and this was found to be effective in treating serious SLE. Less than 20 years ago, routine laboratory tests were developed that helped to measure the amount of disease activity so that physicians could better adjust the doses of prednisone. In the last 15 years, doctors have learned to adjust the dose of prednisone on an individual basis as they have become aware that lupus is a different disease for every person.
Doctors now know that, for many patients, there is a threshold dose of prednisone, which is the lowest dose that a person may take and still keep the SLE under control. For many patients, it may be best to maintain a low dose of prednisone rather than risk a flare.Cyclophosphamide and Azathioprine
Cyclophosphamide (Procytox®) and azathioprine (Imuran®) are powerful drugs which are used in the treatment of certain SLE patients. One of these medications may be used when prednisone alone is unable to control SLE, when prednisone causes too many serious side-effects or when it is thought that combining the drug with prednisone may be better than using prednisone alone. These drugs work in SLE by changing the body's immune response and they are referred to as "immunosuppressive", "immunomodulatory" or "cytotoxic" drugs. Cyclophosphamide and azathioprine are usually given as pills and are generally not used together. As with other kinds of treatment, the decision to use one of these drugs is made on the basis of the symptoms and the condition of the patient. The dosage is based on the patient's weight.
Like all powerful drugs, the immunosuppressives have potential serious side-effects. The three most serious unwanted effects are changes to the bone marrow (where blood cells are produced) which may lead to a decrease in the number of red or white blood cells or platelets, increased risk of infections including infections to which we normally have resistance and a slightly increased risk of developing certain types of cancer. The risk of infection is particularly high when cyclophosphamide or azathioprine are combined with high doses of prednisone. Cyclophosphamide can also be associated with bleeding from the bladder (haemorrhagic cystitis) and failure of the ovaries to produce their normal output of hormones (ovarian failure). In practice, azathioprine is considered less toxic than cyclophosphamide. Obviously, these are very serious problems and patients taking these medications are watched very carefully by their physicians. Periodic blood tests are mandatory throughout the course of this therapy.
These problems have led to a recent report from the American National Institutes of Health on the use of intravenous cyclophosphamide in patients with severe kidney involvement in SLE. In this form of treatment, a much larger dose is given by vein (approximately 10 to 15 times more than the daily dose when given in pill form). By repeating this treatment every 4 to 12 weeks, kidney inflammation was very much reduced as were the serious side-effects (including risk of cancer). Many University Hospital Lupus Clinics in Canada now use this treatment for serious kidney involvement as well as for certain types of serious SLE problems. The procedure can be done on an outpatient basis or during a short stay in hospital. It should be kept in mind that although this form of treatment is better, it is still far from harmless, is used only for SLE patients with very serious disease and that our experience with it is short.
This brings to an end the discussion of the medications that are used in the treatment of SLE. In many patients, these treatments will bring lupus flares under control and the disease may go into remission. It is important to know that you can help keep your lupus from flaring. In the next section, I will tell you about some things you can do and things you can avoid to keep your flares to a minimum.
by Jean-Luc Senécal, MD, FRCPC
Copyright © 1990; Second Edition 1991, 4th printing 1998 Lupus Canada.
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