Dr Ajesh Maharaj
Rheumatologist
Westville - Durban

MB.BS.IMS.BHU(Varanasi);
H.Dip.Int.Med(SA);
FCP(SA) Cert in Rheumatology(SA)

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Systemic Lupus Erythematosis

Introduction

Lupus is a form of arthritis that mainly affects women during their child-bearing years. It is one of the auto-immune rheumatic diseases, caused by a fault in the body’s immune system. The immune system normally produces antibodies to fight infections. In people with autoimmune diseases, antibodies are produced which act against certain body tissues and cause inflammation.

Lupus (Latin for wolf) takes its name from the fact that it can cause serious rashes across the cheeks and nose (rather fancifully resembling the face of a wolf). However, thanks to modern treatment we rarely see these very severe rashes nowadays. There are two main forms of lupus. One, discoid lupus, affects only the ski whereas the other, systemic lupus, involves the joints and may involve the internal organs as well. The full name of this is systemic lupus erythematosus – ‘erythematosus’ simply meaning ‘red’. This article deals only with systemic lupus erythematosus (sometimes shortened as ‘SLE’).

What are the common symptoms of Lupus?

systemic lupus

Lupus can mimic many different diseases. However, it usually starts with joint pains, especially in the small joints of the hands and feet, which may flit from one set of joints to another quite quickly. Skin rashes are also common and these are often made worse when exposed to strong sunlight. You may develop groups of mouth ulcers (not just the one or two that everybody gets from time to time) and these can come back again and again. You may also feel completely exhausted and this can be one of the most common and difficult features of systemic lupus. For many people the disease does not cause symptoms or complications other than tiredness, joint pains and skin rashes. However, in some people it may cause one or more other problems. The challenge facing the doctor looking after people with lupus is to try and identify those who might develop other problems and treat them accordingly. The remainder of this article explains in some detail the nature of lupus and how it might affect some people, mainly with the more severe form of the disease.

Who gets Lupus?

Lupus affects mainly young women. It is much commoner among black women living in Europe and America (though strangely it is rare in Africa) and among Chinese and Asian women. Around one in 250-500 women in Afro-Caribbean origin develop Lupus, compared to one in 1000 in China and one in 4000 Caucasians in New Zealand. It is about nine times as common in women as in men. About one in ten cases begin after the age of 50.

What causes Lupus?

There is no one cause of systemic lupus erythema-tosus. It seems to be due to a combination of factors, including environmental factors (such as previous infections), the balance of hormones in the body, and genetic factors. However, lupus is not an inherited disease – it is very rarely passed on in the family. There are many basic problems affecting the immune system in your body’s self-defence system, made up of cells that patrol throughout the body, in the blood stream, lymphatic system and organs. These cells search out and destroy foreign invaders (such as viruses, bacteria). Unfortunately, when you have systemic lupus erythematosus your immune system cannot distinguish between your own tissue and foreign invaders. It begins to attack your own body. One of the results of this is that proteins called antibodies are formed. Because the body is attacking itself, these are also known as auto-antibodies. They can bind to various parts of your body tissues and in severe forms of lupus can cause damage to various organs including the kidneys, heart, lungs, skin and brain.

Which parts of the body might be affected?

The skin

It is common for a rash to develop over parts of your body which are exposed to the sun; the face, wrists and hands. However, sometimes these rashes can affect other parts of the body. Poor circulation can cause the hands to change colour, with the skin going white, blue and red in the cold. This common problem is known as Raynaud’s phenomenon, after a famous French physician, Maurice Raynaud.

The hair

Loss of hair (alopecia) is common with lupus. This is a minor problem for most people but occasionally you may have quite serious hair loss. As with the other features of this disease hair loss may ‘come and go’.

You will probably find that your hair grows back. You may prefer to wear a wig if you have serious hair loss (though this is rare).

The joints

Unlike rheumatoid arthritis, lupus does not usually cause damage and deformity in the joints although pain and swelling can be the main symptoms of the disease. Perhaps one in every 20 people with lupus develop more severe joint problems which may mimic those of people with rheumatoid arthritis.

The kidneys

Estimates vary but up to one third of people with lupus may develop some inflammation of the kidneys. This does not usually cause problems unless the disease is very severe. Your urine can be tested with a special plastic stick which is coated with various chemicals (a dipstick), This can show if there is protein or blood in the urine. Severe lupus may seriously damage the kidneys but this is rare. This can be treated by modern forms of kidney treatment including dialysis or transplantation.

Blood pressure

Lupus can cause high blood pressure, particularly if the kidneys are involved. Steroid tablets in high dose may also cause an increase in blood pressure. Treatment with drugs to suppress the immune system (see later) can often control and occasionally cure high blood pressure caused by kidney involvement. Also, the various drugs available specifically to control blood pressure are very effective.

The brain and nervous system

You may feel depressed and anxious from time to time. It can be hard to tell whether this is part of the disease itself or simply the normal reaction to having a serious illness. Many people have problems in coming to terms or coping with the disease. It is important to share these feelings with the doctor or other medical professionals looking after you. The medical team can also help you with counselling if you need it. Migraine affects as many as one in three people with lupus. Although lupus can sometimes mimic other neurological conditions, this is rare. However, if lupus does affect the nervous system, it can cause fits or other problems. Often, treatment will control or cure the symptoms so it is important to ask your doctor about any problems you have.

The heart and lungs

Occasionally lupus may affect these organs directly. More often, it inflames the lining tissues around the heart and lungs (the pericardium and pleura). This causes pericarditis or pleurisy with breathlessness and sharp pains in the chest which are worse when taking a deep breath. Rarely, large amounts of fluid may develop in these lining layers, causing severe shortness of breath. This symptom can be treated successfully by removing the fluid.

Other organ involvement

Lupus rarely affects the gut, pancreas, liver or spleen. Lupus may affect the bone marrow and cause anaemia, or a reduction in the number of platelets (which help the blood to clot) or white blood cells. It may, on occasion, cause swelling of the lymph glands, for example in the neck, which may cause discomfort. Some people with lupus have tendency to develop blood clots in veins or arteries. This is usually caused by antiphospholipid and anticardiolipin antibodies. These can also affect pregnancy (see below).

How do doctors diagnose Lupus?

Doctors use a range of tests to check if you have lupus, including:

How can Lupus be treated?

Lupus cannot be cured but it can be controlled.

If you have joint pains and skin rashes you can often be treated with just non-steroidal anti-inflammatory drugs (NSAIDs) and/or an anti-malarial drug, usually hydroxychloroquine (Plaquenil), which is also effective at treating tiredness or exhaustion which can be caused by the disease. Non-steroidal anti-inflammatory drugs can occasionally irritate the lining of the stomach, so it is important for your doctor to arrange regular monitoring of your need for these drugs. There is a considerable debate among doctors as to whether eye tests need to be carried out on a regular basis for people taking Plaquenil. Most doctors continue to ask for these eye tests as a precaution, although very few have ever seen any serious problems due to this drug.

If you have more serious complications, such as pericarditis, you will be given corticosteroids (often called ‘steroids’). These drugs were introduced in the late 1940s and are remarkably effective, sometime life-saving. The dose prescribed will be kept as low as possible to minimise the possible side-effects. These include lowered resistance to infections, osteoporosis, high blood pressure and diabetes.

When lupus is particularly active, it can cause inflamed kidneys, severe anaemia or a very low platelet count. In these cases you will usually be given very high doses of steroids together with other immunosuppressive drugs including azathioprine, cyclophosphamide and possibly cyclosporin (Neoral). These are powerful drugs which need to be carefully monitored with blood and urine tests. The doctor will usually reduce them as quickly as possible as the activity of the disease subsides.

For the most difficult cases of lupus forms of therapy can be used, including the use of intravenous gamma globulin injections and plasma exchange. However, the occasions on which these are needed are rather few and far between.

What are the long-term prospects if I have Lupus?

For many people, lupus is often no more than a nuisance. However, for some people it can be a very troublesome and unpredictable condition. If you have one of the more serious forms of lupus you should be treated by a rheumatologist with an interest in this condition, sometimes in a ‘lupus clinic’. Only careful monitoring over many years will make sure the disease is neither over-nor under-treated, so it essential for all people with lupus to be assessed regularly.

What can I do to help myself?

There are a number of things you can do to help yourself:

Stay out of the sun

Avoid sunbathing. Too much ultra-violet radiation can flare both the skin rash and sometimes the lupus in the internal organs. If you have to go to a hot climate, use a sun-blocking cream, factor 25 or greater. This is available on prescription for people with lupus.

Avoid infections

If you have lupus, then you are more susceptible to infection. Take sensible precautions. For example, if you are already on immunosuppressive drugs, avoid contact with friends and relatives who have infectious diseases.

Watch what you eat

People are very interested in whether diet therapy can help control lupus. Unfortunately, the evidence is very limited. There is more evidence to suggest that a diet low in saturated fat and supplemented by fish oil may be helpful.

Avoid stress

Try to avoid situations which you now ill cause you stress.

Lupus in special situations

Conception and pregnancy

You should use contraceptive pills which contain only progesterone or low dose oestrogen, or consider physical methods of contraception (such as the condom or the cap). If you are given oestrogen after the menopause as a treatment for thin bones (osteoporosis) the risk of a flare developing is slightly increased to about one in seven.

There is conflicting evidence about whether pregnancy itself is more likely to make lupus flare up. However, if your disease is well under control at the time the baby is conceived and you do not have kidney disease you will probably go through pregnancy with little trouble. If you do have kidney disease, then you may well have increased protein in your urine during the later stages of pregnancy. If you have high levels of a particular type of antibody, known as antiphospholipid antibodies, then you are more likely to suffer a miscarriage.

Pregnancy is normally safe, but seek your doctor’s advice before starting a baby.

Only about one in twenty mothers with lupus have particular antibodies to structures known as Ro and La. If you have these antibodies then your baby may have a brief skin rash or a form of heart disease which may require a pacemaker to be fitted shortly after birth.

Doctors are naturally worried about using drugs during pregnancy. It is usually safe to use corticosteroids and there are now many examples of safe use of hydroxychloroquine and azathioprine throughout pregnancy. However, your doctor will try to avoid using these drugs if possible.

Lupus in childhood

Lupus is rather rare in children. It does not usually occur before the age of five, although it has been described in children as young as two. The symptoms and type of organ involvement are somewhat different from those seen in adults – one large recent study showed, for example, that haematological (blood-related) involvement is more common in children. Broadly speaking, though, children with lupus will be treated in much the same way as adults.

Lupus after 50

Relatively few cases (about 10%) begin in this age group and it is often thought that lupus activity begins to decrease after the menopause. However, there is one extra problem for older people with lupus if they are treated with corticosteroids. As mentioned earlier, one of the side-effects of corticosteroids is to cause thinning of the bones (osteoporosis). This can increase the risk of bone fractures as older people are already more likely to have osteoporosis because of their age. Overall, in people over 50, lupus tends to be less severe, but the side-effects of treatment can be more of a problem than in younger people.

What other diseases can affect me if I have Lupus?

Around one in five people with lupus also suffer from Sjögren’s syndrome. This causes dryness of the eyes and mouth, and is named after a Swedish ophthalmologist, Henrik Sjögren. A little under one in ten people with lupus suffer from an autoimmune disease which affects the thyroid gland. This may occasionally make the gland overactive but more usually it makes it underactive. An underactive thyroid gland is often swollen. It causes dry skin, weight gain, and can make you ‘slow down’ mentally. However, all these symptoms are cured by taking thyroid hormone tablets. Much less often, people with lupus may develop rheumatoid arthritis or an inflammation of the muscle (myositis) which causes muscle weakness. Very rarely, other autoimmune diseases may also develop n people with lupus. Most of these additional complications respond well to drug treatment.

Questions and Answers

Is the disease hereditary?

No. Though genetic factors are involved in the development of the disease, may other factors are also involved so it is extremely rare for children of people with lupus to develop the condition.

How much should I rest?

There is no simple answer to this question. When the disease is active, you may not feel like doing very much. When the disease is inactive, you will not gain anything from ‘excessive’ resting. Activity will help to keep your mind off the disease. Many people with lupus do however complain that tiredness is a significant problem. Your body will dictate how much sleep and rest are needed, though some drugs, particularly hydroxychloroquine and steroids, will help to balance the situation.

What about other forms of treatment?

Acupuncture may relieve pain. Like many forms of alternative pain relief, it seems to depend very much on whether you believe it will work.

My hands and feet go blue in the cold weather. Can I do anything about this?

This problem is almost certainly due to Raynaud’s phenomenon which is caused by spasm of the small blood vessels of the hands and feet. Make sure you keep warm and wear thick gloves and socks/tights.

Does stress affect the disease?

Worrying will probably not affect how your lupus develops. However, it will obviously make you feel much worse. Your doctor may be able to help by arranging for you to see a psychologist who will suggest ways of coping designed for you as an individual. This is something which many people find very helpful.

Does the weather make any difference?

The climate will not alter the course of the disease. Some of the worst cases are found in people who live in tropical climates. Although cold, damp weather may make the problem seem worse, it does not really do any specific damage.

Are immunisations safe?

In the main, yes. However, we do not recommend ‘live virus vaccines’ if you are on immunosuppressive drugs. These vaccines include yellow fever, ‘live’ typhoid and oral poliomyelitis. Make sure that you check with your doctor before being immunised.

Summary

Lupus causes troublesome symptoms rather than serious problems in most people. However, effective treatment is available for the vast majority of people with lupus.