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While the dramatic improvement in the outlook for lupus patients during the past few years might be attributed to advances in lupus treatment, it is more probable that the better prognosis is due to the wider recognition of the disease - especially in its milder form - by physicians throughout the world. Nevertheless, there have been significant improvements in lupus treatment.
These lupus treatments relate more to the better use of existing drugs rather than to new "breakthroughs".
- Drugs as Lupus Treatment
- Oral Contraceptives
- Kidney Disease
- Blood Pressure
- Severe Kidney Disease
Drugs as a Lupus Treatment
There is no doubt that many lupus patients have a particularly "allergic" history, developing rashes and other side effects on a variety of drugs. Probably the most common of these are with antibiotics, and sulphonamides (including septrin) and penicillin, in particular, need to be given with care. There is, unfortunately, no way of clearly predicting the person who, for example, will prove penicillin sensitive. Both the patient and doctor need to be highly suspicious of rashes developing within a week or two of starting a new drug. Needless to say, distinguishing between a drug-rash and a lupus rash can be very difficult and should not be left to self-diagnosis.
Drugs used in SLE fall into 4 main groups, being, in ascending order of powerfulness (and dangers of side effects):
For those patients whose symptoms are mainly joint or muscle pain, "traditional" anti-rheumatic treatment is given. Aspirin, though tried and tested, is one of the more indigestible medicines, and in some people, can cause stomach ulceration. It is being largely replaced in most countries by the newer alternatives, so called "non-steroidals". There are over 40 different non-steroidals made - a testimony both to their medical and commercial success. They go under a kaleidoscope of trade names, including Brufen, Motrin, Orudis, Naprosyn, Feldene, Fenopron, Voltarol and so on. These drugs differ only slightly from each other in their actions, as well as in their side effects. It is a well known maxim that though these drugs may appear similar, their efficacy may vary enormously from patient to patient - an observation which continues to baffle drug experts. Despite all the modern tests available, it is obvious that for the individual patient, a certain amount of "trial and error" may be required.
These drugs provide considerable relief, and in some patients, may be the only medication necessary. They are safe, though as with all drugs bringing relief from arthritis, they may cause indigestion.
It is many years since the chloroquine-based group of drugs used in the treatment of malaria were found to help lupus. To this day, the reason why they work in lupus remains largely unknown. The three most widely used drugs are chloroquine phosphate (Nivaquine), Mepacrine (Quinacrine Atabrin) and hydroxychloroquine (Plaquenil) - my own personal favourite. These drugs are particularly useful for those patients with skin and joint disease - and especially those patients with discoid lupus. In some patients, their benefits appear to extend beyond these organs. They may provide sufficient lupus treatment for a patient with moderately active lupus to avoid steroids.
They have a number of minor side effects including indigestion, but they also (particularly chloroquine) have one serious side effect - in prolonged, high dose treatment, these chemicals may deposit in the retina of the eye and, if continued, may impair vision. The implications of this side effect are such that for a long time, these drugs were abandoned in many units.
We now know, however, that with the smaller doses now used (and, with annual eye checks) this risk is very small indeed. Mepacrine (quinacrine), it is thought, may be free from this problem, but it has its own problem - in moderate to high doses it causes yellowish skin pigmentation. Fifty milligrams a day may make the patient look mildly suntanned, but double that does may make the patient look slightly jaundiced - an unacceptable price to pay in many patients.
Common practice is now to prescribe a small dose (eg one tablet of 200mg hydroxychloroquine daily) and to advise the patient that any benefit may be slow in coming (eg 4-6 weeks). If the patient is clearly doing well, after 9-12 months, an attempt is made to reduce the dose - eg to one tablet on alternative days.
Despite the space given here to the problems and side effects of these agents, they are, in my opinion, one of the most useful group of drugs we have in the management of certain types of SLE. It should be noted that Plaquenil is safe in pregnancy.
Steroids, corticosteroids (the most commonly used being prednisolone) have revolutionised SLE. When Hench and his colleagues in America discovered these drugs, and won the Nobel Prize for Medicine, lupus was one of the first conditions in which they were tried. It quickly became clear that their effects were "miraculous", "life-saving". But every drug has its price, and the toll of side effects soon became well-recognised and even awesome.
Lupus quickly established itself as a condition for which steroids were recommended - even imperative. The joint pains eased, pleurisy and pericarditis regressed, the rash lessened, and the fever and general malaise vanished overnight.
These facts remain facts today. The only difference is that, in the light of our knowledge of the long-term as well as the short-term side effects of steroids, our use has become much more cautious.
- Side Effects of Lupus Treatment
What are the side effects of steroids? The list is long and well known to patients and doctors alike. Assuming a moderate to high dose, such as prednisolone 30mg daily over a period of weeks to months, the most noticeable side effects are of increased appetite, weight gain (a higher percentage of ingested food is "kept on board") and a fattening or "mooning" of the face. Other effects, varying in prominence, are sleep disturbance (a tendency to wake at night and sleep by day), indigestion, muscle weakness, and, occasionally, mood disturbance-varying between euphoria and depression.
The long term effects, seen for example in patients who, for various reasons, have been forced to take such doses over a period of years, are much more serious. They include a softening of bone, occasionally leading to collapse of bones such as the vertebrae of the spine or the hip joint, muscle weakness, a rise in blood pressure, and thinning and easy bruising of the skin.
None of these severe side effects is totally irreversible. I have seen many patients who have, on first referral, had the most appalling side effects but, after weaning off the drug, have returned to normal life.
- How do steroids work as a lupus treatment?
Corticosteroids are one group of substances produced normally by the adrenal glands. Even in small amounts, they have general effects on the metabolism of the body, burning protein to form energy giving substances and mobilising sugar. In the larger doses used medically, they have a profound effect on inflammation, suppressing the process. While this may be harmful where inflammation is a normal defense mechanism, in diseases such as lupus, where the inflammation can get out of control, the effect is beneficial, by protecting the body from the ravages of the disease. It is better to have a forest fire smouldering or totally damped down than burning wildly.
- Doses used in lupus treatment
The dose depends on the acuteness or severity of the disease, the laboratory tests, and whether or not delicate organs such as the kidney are involved. In a patient with an active 'flare' of lupus, doses as high as 60mg a day of prednisolone are used. In acute disease, it may be easier to give the steroids "intravenously" (into the vein). The dose is usually 'tailored' down to the minimum required to keep the disease controlled. Steroids cannot be stopped suddenly. The dose must be gradually reduced, in order to allow the patient's own adrenal glands to 'wake-up'. Many physicians change to an 'alternate-day' dosage when the daily level falls to 10 or 15 mg, as this is thought to help the adrenals regain their own function. Many patients are under the impression that, once started, steroids are 'for life'. This is not so - especially not so in lupus, a disease which waxes and wanes. Although it may take a lot of time and patience (some patients find that they cannot tolerate final reductions quicker than lmg per month), there is no reason why, even in those patients who have been on the medication for years, they should not finally be weaned off.
In a disease characterised by an overactive immune system, it might seem logical to suppress immunity, and indeed, over the past two decades, "immunosuppressive" drugs have been used to good effect in SLE. The two most commonly used drugs are azathioprine ('Imuran'), methotrexate and cyclophosphamide ('cytoxan'), though there are many others.
Because these drugs suppress dividing cells, they also suppress the blood-forming cells in the marrow. For this reason, the dose must be carefully monitored with regular blood counts. These agents are generally reserved for patients with active kidney disease, or in those patients whose lupus is requiring excessive doses of steroids. In these limited situations, they have proved very useful.
Newer Lupus Treatment Drugs
In recent years, mycophenolate mofetil ('MMF'; 'cellcept') has become widely used in lupus. Previously mainly used in transplant medicine it is now proving very useful not only in the management of Kidney lupus, but also in other aspects of the disease. One of its virtues is that it is generally well tolerated. Like cyclophosphanide and methotrexate, it cannot be used in pregnancy.
This name is given to a group of new drugs capable of attaching to important sites on specific cells. In the case of Lupus, the so-called B cells are critical, producing as they do, the antibiotics such as anti-DNA, so important in Lupus. Two such "anti-B cell" agents, Rituxmab (Rituxan) and Belimunab (Benlysta) are now available and are being increasingly used worldwide, especially in severe Lupus. As always, price is the limiting factor.
There are theoretical reasons why high-oestrogen oral contraception might be harmful in SLE, and, indeed, one or two patients appear to have first developed their symptoms on starting the "pill". Fortunately, this has not proved to be a common problem, and in the experience of most lupus clinics, there has been little to point to any general harmful effect of the pill. In those patients with migraine or thrombophlebitis, the use of the oral contraceptive is unwise. For the majority of patients with SLE, the use of the low oestrogen contraceptive pill has been without problems.
One of the well-known features of lupus is sensitivity to sunlight. Henrietta Aladjam, who has contributed so much education about lupus, called her book "The Sun is my Enemy".
There is no doubt that sunlight (more specifically UV light) has a major effect in activating the disease. Many patients notice that their rashes, as well as joint pains and other symptoms are worse after a period of sunbathing. Nevertheless, sun sensitivity is not a universal problem, and the majority of patients whose lupus is under control find no special need to avoid sunlight. Sun barrier creams have only been of limited help. Various local lupus groups have collected information from manufacturers in their own countries and have provided considerable guidance in choice of makeup and UV-barrier creams.
Sun sensitive rash on the v-neck area
in a patient with skin Lupus.
This rather ubiquitous heading appears in many articles on lupus. With the evolution of knowledge that has come about concerning lupus, it is clear that for most patients, a normal and active life is entirely possible. Even in those patients going through a long and seemingly endless flare of disease, improvement or 'remission' of disease is likely. The disease does burn itself out.
Faint rash on the back of an infant back of a patient with mild Lupus and "anti-Ro" antibodies.
The rash is casued by the mother's antibody crossing the placenta in pregnancy. It is generally mild and disappear over a few weeks.
Food, its effect on Lupus and Lupus treatment
Patients with most types of rheumatic disease ask about diet. Does food affect lupus? Is there any particular diet which helps? Are there any "special" diets?
Answers to these questions are not quite as straightforward as they once seemed. Although there has been little scientific evidence that dietary input affects any of the rheumatic diseases, there is no doubt that many patients, including a smaller number with lupus, are convinced that certain foods cause an increase in joint symptoms. During the past few years, evidence has been accumulating which suggests that in certain circumstances, food or food products can, after absorption through the bowel, cause an allergic reaction. Obvious examples are some cases of milk allergy in infants. A small number of patients with arthritis have now shown to be worse following certain foods.
Whether the same applies to certain patients with lupus is not certain, though the chances are that it does.
In some patients raised blood pressure becomes a problem. This is most common in those patients with kidney disease, though not confined to this group of patients. It is important to know that raised blood pressure in lupus may be a relatively temporary phenomenon. Nevertheless, of all the features of SLE, this demands perhaps the most meticulous attention.
Unfortunately for the patient, blood pressure treatment usually requires pills - often two or three kinds. The temptation to "throw the pills away" comes to all patients. It is in this situation, where the patient feels fine, that the temptation is strongest. Yet to have a high blood pressure untreated may cause hidden damage, especially to the kidney itself, and a vicious circle of kidney damage - blood pressure, poor kidney circulation - more kidney damage - may occur. All too often, I have been referred to patients whose lupus has been well under control, but in whom the blood pressure has been allowed to run away at too high a level. For most patients with SLE, the diastolic or "lower" blood pressure should normally be kept below 90, and certainly below 100.
The management of those patients who develop kidney involvement in lupus is generally more aggressive, the aim being quick control of disease - albeit at the cost of higher doses of steroids and other drugs. For those who develop "fluid overload", with weight gain and marked ankle swelling, extra medication in the form of 'water pills' or diuretics may be needed, as well as elastic support stockings. Modern fine-material elastic stockings, which have replaced the hideous thick stockings of the past, help to return fluid from the legs to the body.
Occasionally (and rarely these days) patients are diagnosed only when kidney inflammation has progressed insideously to the point of irreversible damage. It was this aspect of the disease which gave lupus its bad reputation in earlier times. Even here, thankfully, lupus treatment is possible with dialysis or kidney transplant. Kidney transplantation is perfectly possible in such patients - interestingly, the lupus itself rarely causes problems after transplantation.
Despite the importance of this aspect of lupus, it seems appropriate to stress its rarity in modern practice. In a personal experience of over 3,000 patients with lupus, I have seen renal transplantation in only two patients - one is now an active housewife, the other a busy cabaret artist in America.
Plasma-pheresis is a technique by which blood is 'cleansed' of potentially damaging products such as certain antibodies. The method is, in theory, simple. Blood from a vein is taken through a tube to a machine rather like a large spin dryer. By centrifugal force, the blood is spun into its various components. The patients red and white cells are returned to the body but instead of the "bad" plasma, a substitute is returned. The method is safe and, apart from the discomfort and boredom of a couple of hours sitting with a blood transfusionline attached to the arm, relatively free of discomfort for the patient. Although this lupus treatment (like the related treatment of leucopheresis, or removal of white cells) is theoretically beneficial, to date the benefits have appeared short lived. This lupus treatment is attractive and has received attention in the world press. However, it has not yet received the benefit of widespread trials and experience.