Yorkshire Lupus Group - Factsheets

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LUPUS UK acknowledges with gratitude the assistance of Dr Graham Hughes (St Thomas' Hospital, London) and Dr Caroline Gordon and colleagues (Queen Elizabeth Hospital, Birmingham) in the provision of clinical information towards the production of these fact sheets.

Note: these Fact Sheets are not intended to be read like the chapters of a book, but individually as appropriate. Some of the material is repeated on several sheets.

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LUPUS fact sheets are available as follows (select link to read the text!):

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Lupus: Incidence within the Community

Systemic lupus erythematosus (SLE or lupus) is a multisystem disease which can affect people of all ages and has been found worldwide, although some groups of people are at greater risk of developing the disease than others (see below). The clinical features of the disease and the tests used to diagnose it are discussed in other factsheets. This one covers studies estimating the incidence of the disease (how many people develop the disease) and the prevalence of the disease (how many people have the disease) at a particular time. In other words, it covers 'who gets the disease'. 'Why they get the disease' is harder to answer but will be discussed briefly.

How common is Lupus?

Until a few years ago there was very little information on how common lupus is in the UK. It was generally considered a rare disease, most general practitioners (GPs) only having one or two patients on their lists. However, studies done in the late 1980s and early 1980s have shown that lupus is more common then was realised, particularly in women. Results from the largest UK study in Birmingham showed that in a population of 1.2 million, there were 242 adult people known to have lupus and 33 new cases of lupus diagnosed in one year (1991; children were not studied). From these figures the prevalence and incidence rates for lupus (corrected for the age of the population) were calculated. The prevalence was 28 per 100,000, that is about one person in 3,500 has lupus. The incidence (new cases per year) was 3.8 per 100,000, that is about one person in 26,300 developed the disease that year. The previous results from the smaller cities of Nottingham and Leicester were very similar.

What groups of people are most at risk of lupus?

Many studies have shown that women are about 10 times more likely to have or to develop lupus than men. In Birmingham, the figures showed that lupus occurred in almost exactly one in 2,500 adult women; which shows that lupus is not a rare disease in women. It is however rare in men, occurring in approximately 1 in 25,000 adult men. Although the disease can start at any age, the first signs of the disease in women usually appear during the reproductive years (after the onset of menstrual periods and before the menopause). The disease is most commonly diagnosed in women between the ages of 20 and 40, and it appears to be milder in those in whom the disease starts after the menopause (when the monthly periods stop). There is no particular age pattern in men with lupus.

It is also well recognised that people from different ethnic and racial backgrounds are at different risks of developing lupus. People of West Indian origin are particularly likely to develop the disease, even when they are born and live in North America or the UK. Surprisingly, people of West African origin (from which the West Indian populations were descended) are at low risk of developing lupus. Studies have suggested that up to 1 in 250 women in Jamaica develop lupus. In Birmingham, 1 in 500 women of Afro-Caribbean background have lupus, compared with about 1 in 1000 women from India and Pakistan, and about 1 in 2,500 white European Caucasians. Other studies have shown that people of Chinese and Polynesian backgrounds are also at increased risk of developing lupus, compared with white Caucasians.

Why do people get lupus?

These observations on the different risks of developing lupus in different populations have suggested that genetic factors (which people are born with), play an important role in the development of the disease. This does not rule out a role for environmental factors which may also be shared by people from particular backgrounds. There is no single gene which puts people at risk of developing lupus (unlike haemophilia and cystic fibrosis). It seems most likely that between 20 and 80 genes contribute to the risk of lupus and that the genes set the scene and environmental factors contribute to whether or not the disease develops and when. The 'environmental' factors include exposure to UV light (sun-exposure), various infections, possibly chemicals in the environment, factors related to stress (not well-identified) and female hormonal activity (for example the oestrogen-containing contraceptive pill or pregnancy). These factors combine together to influence the immune system in such a way that immune abnormalities result which cause the disease to develop (or recur).

Will children of people with lupus get lupus?

Because so many genes are involved in the development of the disease (only half of which are inherited by a child from one parent with lupus), and because environmental triggers have to occur during the life of the individual to make the disease appear, it is not common for the children of people with lupus to have lupus. Most studies have shown that about 1 in 20 people with lupus will have a close relative (mother, aunt, sister, brother; less often father or uncle) with lupus. Occasionally the baby of a mother with lupus will be born with a special form of lupus called neonatal lupus syndrome, due to the passage of certain antibodies (anti-ro and/or anti-la) from the mother to the baby during pregnancy. This form of lupus only lasts a few months, as the baby destroys the antibodies from the mother and does not make any more itself. It does not predispose to lupus in the future in that child.

Are there cases of lupus in the community which have not been recognised?

Lupus can be difficult to recognise because the symptoms overlap with those of other conditions. Although the typical sun-induced rashes are often picked up by GPs and hospital doctors, some of the other manifestations may not be recognised unless they all occur at the same time and the appropriate blood tests are done to help confirm the diagnosis (see fact sheet on Lupus and Laboratory Tests). In Birmingham, a study was done to look for undiagnosed cases that were not picked up in the survey mentioned above. A questionnaire looking for lupus symptoms was sent to a group of women and those with a certain number of positive answers were asked to have a blood test (the ANA test). If it was positive, the people were asked to attend for a clinical assessment by a specialist doctor and more blood tests. In this way three new cases of lupus, out of 3,000 adult women sent the questionnaire, were identified. If the results of this small study were repeated on a larger scale, it could mean that up to 1 in 500 adult women (not 1 in 3,500) have lupus. This is probably an overestimate, but it is quite possible that there is a number of undiagnosed cases in the community.

What should I do if I think I have got lupus?

If you think you have lupus after reading LUPUS UK fact sheets, you should see your GP and discuss why you think you have SLE and show the GP the fact sheets if necessary. If the GP agrees that lupus is possible, the GP may arrange the blood tests or may ask for a second opinion from a doctor at the local hospital. If the initial blood results support the diagnosis, a specialist opinion on the need for further tests and treatment is recommended, as lupus is a complex and variable disease. Most people do well with appropriate treatment but there is presently no cure, and lifelong follow-up of the disease is required.

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Lupus: A Guide for Parents

Lupus is a disease where the immune system becomes overactive. Antibodies are produced and almost all organs of the body can be involved.

Who gets lupus?

Lupus is most common in young females (teens, 20s and 30s). Men and children can also have lupus. The ratio of females to males is nine to one.

Is it a world-wide disease?

Yes, lupus affects people from all countries in the world. There are certain countries in which the disease appears to be more prevalent, for instance the Caribbean, the Far East and China.

What is the outlook?

Most patients with lupus can expect a normal life-span. The disease, if diagnosed early and treated appropriately at an early stage, most commonly settles and ultimately goes into remission - i.e. the patient requires no medication.

What are the features of lupus?

Fatigue, flu-like illness, skin rashes (including the classical 'butterfly' rash on the cheeks and nose), hair-loss and, more importantly, internal organ involvement including pleurisy, kidney disease and brain inflammation. Some patients with lupus have a clotting tendency and this can present, for instance, as a thrombosis in the vein or an artery.

How is it treated?

Early in the disease, usually in the more aggressive stages, it is treated with corticosteroids and/or antimalarials. Where there is kidney or brain disease other drugs such as immunosuppressives are used. In those patients with a clotting tendency anticoagulants are used. Management is usually aimed at reducing medication to the lowest dose possible and, ultimately, to weaning the patient off stronger medication.

How can I help myself?

Some of the factors which exacerbate lupus are stress, excessive sunlight and, occasionally, drug allergies (especially to the antibiotic Septrin). Once the patient is treated there is every possibility of a normal lifestyle. Even those patients who are sensitive to ultraviolet light and develop skin rashes can, with normal commonsense protection against excess UV exposure, lead a reasonably normal lifestyle. There are no diets which have been found especially helpful in lupus though it must be said that some patients do appear to be allergic to certain foods and the only way to find this out is by trial and error.

Where can I find out more?

In addition to the self-help societies such as LUPUS UK there are patient networks in most countries of the world. For doctors researching lupus, there is an international journal 'LUPUS' as well as a growing number of books available on the disease.

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Lupus: the symptoms and diagnosis

Fatigue

This is one of the most common and certainly one of the most prominent features of lupus. Patients often describe it as an unnatural fatigue. Its causes are not well understood. Often it precedes the diagnosis by months or years and only when treatment has been successfully started does the patient realise how major a feature it had been.

Aches and pains

The majority of lupus patients suffer at some stage from joint and muscle pains. In many patients this presents as 'pain all over'. In acute flares of lupus the symptoms are often described as being 'flu-like'. Unlike other rheumatic diseases such as rheumatoid arthritis, there is often very little to see in the way of joint swelling. It is not just the joints that are affected but the tendons and muscles as well. In the majority of cases the joint inflammation does not progress to permanent damage.

Fevers

Fever is usually a feature of a flare of the disease. Fever is unusual when the disease is in a quiet phase: thus in an adult or a child known to have lupus who develops fever the possibility that a separate diagnosis - infection - might be present always needs consideration.

Rashes

A wide variety of skin rashes occur in lupus. Traditionally these are sun-sensitive (photosensitive) but this is not always the case. The commonest rashes are on the cheeks (the butterfly rash across the nose and cheeks), on the elbows, on the palms and soles and on the V-neck area. The rashes vary from pinkish discolouration through to blisters and small pinpoint 'blood spots' (purpura). Most rashes in lupus have a tendency to come and go.

Hair loss

Hair loss is one of the most important features of active lupus. It may be the first manifestation of the disease and is often first noticed by the patient as hair on the pillow. In some cases hair loss is patchy and even extreme. Fortunately, in the vast majority of patients the hair re-grows after successful treatment, though hair regeneration is often notoriously slow.

Headaches

Headaches are a major feature of lupus. In some patients a history of headaches or a typical migraine go back to the patient's teens and pre-date the diagnosis by many, many years. There is almost certainly a variety of causes of headaches in systemic lupus. One specific and important cause is 'sticky blood' caused by the presence of antiphospholipid antibodies (see the fact sheet on antiphospholipid syndrome or Hughes' syndrome).

Depression

Depression is an important feature of lupus. It is sometimes simply attributed to 'being unwell' or having tiredness and pain. However, in many patients it is far more important than this and is a primary feature of the disease. It sometimes responds well to management of the lupus itself and is clearly a central feature of the lupus process. In some patients the return of depression is a tell-tale sign that the lupus is flaring.

General symptoms

As almost every organ in the body may be affected at some time, the symptoms and signs are legion and can include irritation of the eyes (sometimes associated with dry eyes), mouth ulcers, chest pain (pleurisy is, for example, important in active lupus), weight loss and ankle swelling. Some of these features will be discussed in other fact sheets when individual organs are being reviewed.

Diagnosis

The diagnosis of lupus is usually made on clinical grounds. The combination of some of the features described above, especially the skin rashes, usually but not always makes the diagnosis clear. Unfortunately, in many patients, especially those who do not have the classical tell-tale rashes, the diagnosis is missed. This is particularly true for those with more 'vague' symptoms such as fatigue, depression or headaches. Often the patients are given the wrong diagnosis such as ME or 'atypical multiple sclerosis'. Diagnosis is critical and any individual in whom lupus is suspected (or for that matter the relative or offspring of any individual with lupus in whom the diagnosis is a consideration) should have the simple blood tests performed.

Lupus blood tests

Lupus is now almost invariably diagnosed by blood tests. These invoke a small amount of blood and are extremely sensitive. There are five major blood tests carried out on the blood sample.

  1. Antinuclear antibody (ANA). This cheap and reproducible test is the 'screening test' for lupus, being positive in over 90% of cases. It is not specific for lupus but because of its simplicity is a useful first step in diagnosis.
  2. DNA antibodies. This is the highly specific test for lupus. For some unknown reason the presence of antibodies against double-stranded DNA is the hallmark of lupus. It is very specific for this disease and rarely found in any other condition. Strongly positive anti-DNA antibody tests provide almost total proof of the diagnosis. The level or titre of the antibodies provides a rough guide to disease activity and is used by physicians to monitor the ups-and-downs of the disease.
  3. ENA. The term 'extractable nuclear antigens' applies to a battery of other antibodies which are found in lupus variants such as Sjogren's syndrome and mixed connective tissue disease - these will be discussed in a separate fact sheet.
  4. Antiphospholipid antibodies. These tests are associated with the important problem of 'sticky blood'. Patients with high levels of antiphospholipid antibodies have an increased tendency to clotting both in the veins and arteries, and in pregnant women with these antibodies there is a risk of thrombosis of the placenta leading to miscarriage. It is now recognised that many women with recurrent miscarriages have antiphospholipid antibodies and that successful pregnancies are possible when the patient with sticky blood is treated either with aspirin or with an anticoagulant.
  5. Complement. This is a term used for a group of proteins in the blood which are involved in the immune process. In active lupus the levels of complement (usually measured as C3 and C4) are low and these often provide a clue to the degree of disease activity.
  6. General blood tests.

    In addition to the specific blood tests, the physician usually requests a full blood count and biochemistry. The blood count in lupus can show low white cells, low red cells and low platelet counts. Biochemical tests are important, especially the creatinine and urea which are raised if there has been evidence of kidney disease. Two blood tests, the ESR and the C-reactive protein (CRP) are used as barometers of disease activity.

Urine tests

Testing the urine is vital in lupus patients and it is the practice in some lupus clinics to teach all patients how to test their own urine. The simple test uses a 'dip-stick' to check for protein - often the earliest clue to the presence of kidney disease. More precise urine tests are performed on a MSU (mid-stream urine - a sample of urine sent to the laboratory for microscopic analysis). Under the microscope, the presence of white cells, red cells or clumps of cells (casts) is recorded - all possible signs of kidney disease. Finally, all urine sent to the laboratory is tested for bacterial infection.

More complicated tests

The lupus patient may require specialised tests to look for more widespread organ involvement. These will include echocardiograms, brain scan (NM), kidney scans and, if there is evidence that the kidney is inflamed, possibly a kidney biopsy. Having said this, for the majority of lupus patients attending routine lupus clinics, a simple blood test and urine test are the basic requirements. From these two analyses a broad picture of the degree of lupus activity can be readily obtained.

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Lupus: the heart and lungs

Symptoms and signs

The main symptoms and signs of heart or lung involvement are chest pain (sometimes on deep breathing-in), shortness of breath, cough, and ankle swelling. The commonest manifestations of lupus in the heart and lungs are in the linings of these organs, the pleura and pericardium.

Pleurisy

Pleurisy is common in lupus - estimates vary between 30 and 60% of all patients at some time suffering from inflammation of the lining of the chest. The symptom is pain - often a 'catching' pain on taking a deep breath. More severe forms of inflammation and pleurisy produce fluid, so-called pleural effusions. Any fluid in the chest takes useful breathing space away, therefore causing shortness of breath. Pleurisy can be detected clinically and on chest X-ray. Fortunately, it usually responds rapidly to a short course of steroids.

The lungs

Other lung conditions are less common than involvement of the linings of the lungs (pleura). In a small number of patients a scarring occurs in the lungs, so-called fibrosis. This can be detected both clinically or on chest X-ray or scan. It produces shortness of breath, especially on exercise. Another rare manifestation of lupus in the lung is the so-called 'shrinking lung' where on chest X-ray the lung areas look smaller than normal. These are all signs of active disease and require treatment.

The heart - Pericarditis

The pericardium or tissue membrane surrounding the heart is identical to the pleura surrounding the lungs and is also frequently inflamed. Classically, the pain is in the centre of the chest at the front and sometimes mistaken by patient and doctor for a heart attack. It can be detected clinically or on chest X-ray or echocardiogram. Fortunately, it rarely causes damage to the underlying heart and responds to steroids.

Heart valve disease

A small number of lupus patients develop heart valve disease. Many of these patients were, in the old days, wrongly diagnosed as rheumatic fever, but we now know that this was not the case. There is a very strong association with the presence of antiphospholipid antibodies ('sticky blood') and this will be discussed in another fact sheet. Leaky heart valves result in shortness of breath and require expert cardiological advice. Ultimately some patients require valve surgery. Fortunately this is exceptionally rare.

Heart attacks

It was discovered some years ago that a number of lupus patients who have seemingly recovered from their lupus still suffer from coronary disease and have an increased chance of a heart attack. The reasons for this are not clearly known although considerable research work is now going on to look at possible risks. Certainly, steroids have been implicated although they are not the major culprit. Some patients with lupus have persistently raised cholesterol and other lipids. Modern treatment is very effective in reducing these. Most recently antiphospholipid antibodies have been implicated in the clotting process in coronary arteries as well as in other arteries and it may well be that taking regular blood-thinning medicines such as low-dose aspirin will be shown to reduce this risk.

Conclusions

The heart and lungs are not the most commonly affected organs in lupus but pericarditis and pleurisy are often extremely distressing. Fortunately they respond well to steroid treatment. More emphasis is now being placed on looking at risk factors for coronary artery disease - risk factors such as abnormal clotting profiles and abnormal cholesterol levels.

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Lupus and the Brain

Doctors throughout the world are now recognising the importance of subtle forms of brain involvement in lupus as well as the more obvious brain problems. Never underestimate brain involvement. It may vary from mild depression, to memory loss, to much more severe problems such as seizures. In general there are two main causes of brain disease in lupus. The first is lupus disease itself which can cause alterations in the brain activity. The second is the clotting disorder associated with some lupus patients, the antiphospholipid or Hughes syndrome. It is very, very important for the doctor to try to distinguish between these two major causes of neurologic involvement as the treatments are clearly very different.

Depression

Depression is an important manifestation of lupus - in some it is the presenting sign of the disease. Many patients and, certainly, many doctors wrongly attribute depression in lupus merely to having a chronic illness and all that goes with it. This is not correct. The disease itself causes depression. Depression is an integral part of lupus in some patients - indeed management of the lupus often itself lifts the depression. The management of depression in lupus rests on a combination of treating the underlying lupus itself as well as possibly adding in antidepressant therapy. One of the medical advances in the last decade has been the introduction of newer milder antidepressants with less of the severe side-effects which so hampered older treatments.

Headaches

Headaches are common in lupus. In some patients a history of headache going back to their early teens is a feature of the disease. They may be a part of the lupus itself or may be associated with a clotting syndrome. They may or may not have a migrainous element with flashing lights and visual disturbances. In any patient with lupus who suffers from headaches a systematic search for known causes should be carried out including blood pressure checking, examination of the sinuses, examination of the blood for 'sticky blood' and ultimately, if indicated, a brain scan (either an NM scan or a CT scan).

Fits

Sometimes lupus first starts in the most dramatic way with a seizure or a series of epileptic fits. This is usually when the patient is untreated and the disease fairly active. It is sometimes associated with high fever. Fits or seizures are one of the non-specific ways the brain reacts to severe illness. Once the lupus is treated further fits are the exception rather than the rule.

Movement disorders

The same applies to movement disorders. Occasionally patients develop chorea (St Vitus Dance) with jerky hand movements or head movements. This is simply a manifestation of abnormal brain function and, once again, is often associated with the 'sticky blood' (Hughes) syndrome.

Spinal chord

Rare, but extremely acute and very dangerous, is spinal cord involvement, which may lead to permanent paralysis. It is now recognised that immediate treatment with both steroids and possibly anticoagulants may reverse this. Fortunately it is a very rare manifestation of neurologic lupus.

Psychiatric disturbance

During severe lupus flares patients can experience a variety of psychiatric disorders varying from mild personality disorders to severe psychotic behaviour. Some lupus patients are wrongly diagnosed as having schizophrenia at the onset of their illness. Interestingly, treatment of the lupus in these patients results in total improvement in the psychiatric features. This is one of the most important observations to come out of lupus research as it provides possible insights into other mental disease. Patients with the antiphospholipid (sticky blood) syndrome suffer memory variants, from subtle ('I couldn't remember what I had gone into the shop for') to severe memory loss. Lupus doctors are now beginning to realise how common and important this aspect of the disease is. Clearly, any patient who feels that this is a major feature of the disease requires full neurologic examination, possibly including MRI, as well as testing for the antiphospholipid syndrome.

Treatment

Firstly, it is important to realise that brain involvement in lupus is extremely common. Secondly, in the vast majority of patients there is complete resolution of the problem with time and most patients get better. If the brain symptoms start dramatically, for example with fits or severe neuropsychiatric disease, the treatment, as with most active forms of lupus, is with steroids and immunosuppressive drugs. The doses of steroids used are less than in the old days - for example 60mg daily in the majority of the worst cases - rarely is a higher dose than this required. An alternative way of giving steroids is by 'pulse' injections on an intermittent basis. This is becoming more popular as it is a simple and more rapidly effective way of giving steroids, especially in an emergency.

A separate form of brain involvement in lupus is associated with Hughes syndrome (the antiphospholipid syndrome). In this form of the disease the cause is totally different: blood clots or 'sticky blood'. In patients where this is suspected, brain scans are usually required. These may show localised areas where brain blood supply has not been adequate. The treatment in these patients is different and requires thinning of the blood, either with aspirin or, in more severe cases, with anticoagulants such as warfarin (coumadin).

For less dramatic brain involvement the choice of treatment in many ways is more difficult. Many, many patients are not treated who perhaps should be treated. In some patients the depression is a major problem and requires conventional antidepressive treatment. The more modern pills for depression are very superior to older medications and cause far less side-effects. The opinion of a psychiatrist or psychologist may need to be sought as to whether medical treatment is appropriate, especially where there might be dangers of drugs interacting.

In summary, the vast majority of patients who have brain involvement can be treated successfully with a full return to normal daily activities.

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Lupus and the Kidneys

Together with the brain the kidney is potentially the most serious organ involved in lupus. Serious in that it may be 'silently' involved - the patient not knowing that there is disease going on, The early diagnosis of lupus in patients throughout the world has contributed more than anything else to the improved prognosis. It is now known that if caught in time kidney inflammation can be treated successfully.

How frequent is kidney involvement?

Estimates vary depending on the type of clinic and the patients studied but it is usually said that approximately half of all lupus patients at some stage have clinical evidence of kidney inflammation. It may be that with a diagnosis of milder cases of lupus this percentage will fall. Fortunately, extreme kidney disease requiring kidney dialysis and even transplantation is extremely rare in lupus.

Symptoms and signs

Kidney involvement in lupus rarely causes discomfort or pain (as distinct, for example, from kidney stones or infection). The most common major kidney problem is that of protein leakage in the urine. This can be mild and detected only on testing, or severe gradually leading to a lowering of the protein level in the blood (a low albumin level). When this happens there is a tendency to ankle swelling, to fluid retention and to general fluid bloating.

When the kidney is inflamed the blood pressure frequently rises and blood pressure measurement is one of the important parts of the physical examination of lupus patients. When the kidney is more severely damaged its normal filtering process is grossly impaired and toxic elements such as urea and creatinine, normally present in the blood in small amounts, build up leading to weight loss, nausea and general malaise.

Urine testing

Simple 'outpatient' urine testing involves the use of a dipstick. Modern urine testing sticks test for a variety of constituents in the urine including urine sugar, albumin, bile products and so on. The test simply involves the dipping of the stick in the urine and comparing the colour changes with a colour chart. If the lupus patient is losing protein in the urine ('proteinuria') then the amount may need to be quantified. For this a 24-hour urine preparation is required. All the urine over a full 24-hour period is collected and the precise amount of protein lost over this period is measured. For other urine constituents the sample is sent to the laboratory for analysis for bacteria and for microscopic examination. Normal urine under the microscope is clear but when there is inflammation anywhere in the urinary tract (in the kidneys or the bladder) cells are present, either red cells or white cells. More important is the presence of clumps of cells called 'casts'. These clumps - looking rather like a railway train of goods wagons - is indicative of kidney inflammation rather than bladder inflammation and is of vital importance in the diagnosis and assessment of the kidney.

What the blood tests show

Much information concerning kidney function is obtained from simple blood tests. The three main blood tests affected by kidney function are the blood urea (sometimes called blood urea nitrogen or BUN), the creatinine and the albumin. If the vital filtering function of the kidney is impaired then urea and creatinine levels start to rise and these two measurements are the most important guides to the severity of kidney involvement. The blood level of albumin (protein) falls if leakage of the protein in the urine is present.

In addition to these three tests a number of other blood tests give important information. These include the sodium, potassium and calcium levels and the blood haemoglobin -all directly or indirectly affected by altered kidney function.

More complicated tests

More precise tests of kidney filtering function consist of a creatinine clearance test (another test involves a 24 hour urine measurement}, an updated and more precise radio-isotope study called EDTA clearance and, to determine kidney size, ultrasound.

Kidney biopsy

In some patients the only way of determining precisely the degree of disease activity is to perform a kidney biopsy. This is now a routine procedure in hospitals throughout the world. It is most safely carried out under X-ray scanning. Following a local anaesthetic given in the loin a needle is inserted into the kidney and a small core is obtained. The patient is usually kept in hospital overnight as there is a small risk of bleeding following biopsy. The procedure has a very high safety margin and does not adversely affect kidney function. The interpretation of the kidney biopsy by the pathologist takes a lot of expertise. Put at its most simple the first signs are these of inflammation (cells are seen around the filters). The second and more serious stage is damage to the filters (glomeruli). The most severe stage is when all the glomeruli are scarred. There are international conventions about 'staging' the severity of the kidney biopsy and pathologists are able to judge the chances of response to treatment from their reading of the biopsy.

General treatment

It is now widely agreed that when there is kidney inflammation a combination of steroids and an immunosuppressive medicine is required. For active or severe kidney disease the most widely used immunosuppressive is cyclophosphamide given intermittently by injection. In the olden days cyclophosphamide was given as a tablet but this produced more side-effects and most units have now converted to giving intermittent 'pulses'. This comes in the form of a drip given into the vein, usually given weekly for three weeks then monthly for 3-12 months. Doses vary from clinic to clinic but the more modern fashion has been to use lower doses than those previously used and this has the benefit of far less side-effects. These side-effects will be discussed in another fact sheet on treatment. A milder and very widely used immunosuppressive is azathioprine given as tablet-form usually at a dose of about 2'mg/kg body weight. All immunosuppressives can affect the blood count and regular blood counts are mandatory. Other immunosuppressive drugs such as cyclosporin-A are increasingly used but the two mainstays of treatment remain cyclophosphamide and azathioprine.

Is dialysis helpful?

If the kidney damage reaches a stage where toxic chemicals build up then dialysis is vital. Dialysis has been one of the major advances in 20th century medicine and either haemodialysis or peritoneal dialysis has kept thousands of patients with renal failure stable. This includes a number of patients with lupus.

Does renal transplant work?

The answer is very definitely yes. One of the surprises in the early days of transplantation in lupus was that the lupus did not return to damage the transplanted kidney. The reasons for this are obscure, possibly related to the strong treatment used for transplantation but possibly to other factors. It is a striking fact that patients with lupus who do have renal transplantation in general do very well indeed.

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Lupus: the joints and muscles

Joint and muscle pain is one of the commonest symptoms experienced by people with lupus and most people will suffer from some such problems during their illness. This fact sheet wlll discuss some of the problems encountered and their treatment.

What happens to the joints in lupus?

In lupus the joints can become inflamed causing pain and swelling. The joints most frequently involved in this inflammation or arthritis are the hand joints, the wrists and the knees, although any joint can be involved. The arthritis frequently comes and goes and affects different joints at different times. The ligaments and tendons around the joints can also become inflamed and tender. If the inflammation is not brought under control with medication and continues for a long period of time, the tendons and ligaments can weaken. Once this happens the tendons and ligaments can no longer support the joint properly The affected joint becomes lax, or unstable, and can appear to be deformed. The hand joints are the most frequently affected by such deformities. The underlying bones themselves are not affected by the arthritis in lupus, and at least initially the deformities can be painlessly corrected by pushing the joint back into position.

What treatment is available for the joints in lupus?

Sometimes painkillers, such as paracetamol or coproxamol, are all that are needed to control the joint pain. If this is not enough then the addition of an anti-inflammatory such as ibuprofen or diclofenac can improve the pain and swelling. If there are just one or two troublesome joints an injection of steroid into the particular joints may be recommended; this is often an effective way getting the maximum benefit of the steroids without much risk of side effects. If there are more joints affected than could easily be injected then steroids may given into the muscle (intramuscular) or directly into the vein (intravenous). Intramuscular and intravenous steroids can result in a rapid and dramatic reduction in pain and inflammation of the joints. However the effect is often short lived and the treatment usually needs to be supplemented by some oral medication.

The commonest oral medication to be recommended are the antimalarials, hydroxychloroquine and chloroquine. Both of these are effective in reducing joint pain and inflammation over a long period of time, but they can take up to 3 months to become effecilve. Steroids by mouth are also effectlve in controlling joint pain but because of their long-term side effects they are usually held in reserve. Sometimes joint pain and inflammation can be particularly troublesome and stronger drugs such as azathioprine, methotrexate and cyclosporin are needed to control the arthritis.

What about surgery?

Surgery can be helpful for some people. Hand surgeons can correct some of the hand deformities with operations on the tendons, ligaments and joints. Orthopaedic surgeons can replace some of the larger joints, for example knees and hips, if they are particularly badly damaged. Surgery is a big undertaking in people with lupus and the disease needs to be well controlled to make the procedure as safe as possible and to increase the likelihood of a good result from the operation.

How does lupus affect the muscles?

Lupus can affect the muscles in a number of ways. The commonest cause of muscle pain is related to arthritis in nearby joints and in this case treating the joints also helps the muscle pain. An infrequent but serious cause of muscle pain in lupus is direct inflammation of the muscles or myositis. Weakness is often more of a problem than pain in myositis, and this can be a serious problem when the muscles that control breathing and swallowing are affected. Myositis is diagnosed by a number of tests including blood tests, electrical testing of the muscles and a muscle biopsy where a small sample of muscle is taken usually from the thigh. A less serious but more common muscle problem is a condition called fibromyalgia. Fibromyalgia can occur in pedple both with and without lupus. It causes persistent pain in most muscles but tends to be centred around the shoulders and hips. It causes sleep disturbance and tender spots in the muscles can develop. The causes of fibromyalgia are unknown, but thankfully it does not go on to cause muscle or joint destruction although it can cause considerable discomfort.

How can myositis be treated?

Myositis although a serious problem usually responds well to treatment with steroids; depending on how ill the person is these may need to be given intravenously. Other drugs are frequently added to the steroids to improve or maintain the condition including azathioprine and cyclosporin. In life threatening cases cyclophosphamide and gamma-globulin (an intravenous treatment consisting of human antibodies) can be used.

How can fibromyalgia be treated?

Fibromyalgia, although not as serious as myositis, can be a difficult problem to treat. It is known that the less mobile a person becomes with fibromyalgia, then the more painful the joints become. Therefore the first line of attack is to get the people doing some exercise either on their own or under the supervision of a physiotherapist. Although this often causes more pain to start with if the person persists the pain diminishes and sleep improves. If these simple measures are not enough drug therapy can be helpful. The drugs most commonly used are the antidepressants. In low doses these drugs do not act as an anti-depressant but have beneficial effects on sleep pattems and can help reduce pain. The most frequently used drug is amitriptyline although there are many different ones available.

Are there any other joint or muscle problems in lupus?

Long-term treatment with drugs can cause problems to the joints and muscles. The most well known problems are with the steroids. Steroids over a long period of time can cause thinning of the bones, osteoporosis, and increase the risk of fractures. Steroids can also have effects on the muscles causing weakness and a similar condition to myositis. Steroids have also been linked to a condition called avascular necrosis of bone. In this condition the blood supply to certain areas of bone (usually the hips and shoulders), is reduced, causing the bone to die and the joint to crumble. The only treatment available for avascular necrosis is joint replacement. Doctors are more aware of the problems of steroids than in previous years and try to keep the dose of steroids low enough to keep the lupus under control but reduce the occurrence of side effects. Some of the other drugs used in lupus can have effects on the joints and muscles including methotrexate and cyclosporin but these effects tend to be rare.

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Lupus: the skin and hair

Skin problems are very common in lupus and many different types of problems can occur. This fact sheet will discuss some of the more frequent problems encountered by lupus sufferers.

What types of skin problem occur in lupus?

There are many different skin problems in lupus, which vary from person to person. The 'classical' skin problem in lupus is the butterfly rash. This is a red rash, sometimes no more than a mild blush, that occurs across the bridge of the nose and on the cheeks resulting in a distinctive butterfly-like appearance. This type of rash tends to occur in the systemic form of the disease where other organs in the body are also involved. It is often present at the beginning of the disease, helping doctors make the diagnosis. Butterfly rashes tend to come and go depending on how active the underlying lupus is but it does not leave scars as it heals.

Discoid lupus is a type of lupus that tends to be confined to the skin and other organs in the body are not involved. Discoid lupus occurs in patches across the body. These patches tend to be well defined, thickened and scaly, they are slightly red in colour and can itch. The appearance can vary between people and also on different areas of the body on the same person. As the patches heal they tend to leave scars and on darker skins the pigment in the skin can be lost leaving white areas. If discoid lupus occurs on the scalp the hair will be lost as the patches heal leaving permanent bald areas.

Subacute cutaneous lupus is a distinct rash that usually occurs in sun-exposed areas of the body. It starts as scaly patches which increase in size to form circular areas which gradually heal up without leaving scars. This type of lupus falls in-between the systemic form and the discoid form: people with subacute cutaneous lupus often have some of the blood abnormalities found in systemic lupus and frequently joint pains, but they do not usually develop the serious complications that can occur in the systemic disease.

Practically any type of rash can occur in systemic lupus ranging from widespread mild rashes similar to those seen in viral illnesses such as German measles, to small distinct patches of rash on the elbows and knees resembling another skin condition, psoriasis. Two particular problems that can occur are panniculitis and urticaria. Panniculilis is inflammation of the fat below the skin resulting in tender red lumps beneath the surface of the skin; these heal slowly over time and can leave dimpling of the skin when fully healed. Urticaria is an itchy raised red rash similar to nettle rash that can occur with vasculitis (see below) or on its own; it heals without leaving scars.

What about the blood vessels in the skin?

Lupus can affect the blood vessels in the skin. The blood vessels themselves can become inflamed. This is known as vasculitis. Vasculitis can cause painful red spots frequently on the hands and feat and sometimes chilblain type rashes. Vasculitis can also occur in other areas of the body for example the kidney which can be very serious and requires prompt treatment The blood flow through the skin blood vessels can become sluggish in lupus patients who have proteins that affect the clotting of the blood known as the antiphospholipid antibody syndrome. In these people the skin may take on a mottled net-like appearance known as livedo reticularis usually on the legs and the arms.

What happens to the hair in Lupus?

The hair often thins and can become patchy when lupus is active. It will usually regrow as the disease is bought under control. This is not the case in discoid lupus as already discussed when the scars left by the skin rash leave permanent bald areas. Sometime drug treatment can make the hair thin in lupus patients. This is known to happen in some people with steroid treatment and in most people when cytotoxic drugs such as cyclophosphamide are used. In both cases the hair should regrow when the drug is discontinued.

What about sunlight and the skin in Lupus?

Approximately 60% of people with Lupus will be sensitive to the sun. Sunlight can cause an exacerbation of skin rashes, burning of the skin and increased activity of lupus in other organs within the body. Some people will also be sensitive to certain types of lighting inside buildings such as fluorescent tubes and high intensity lighting. It is not clear why some lupus sufferers are sensitive to the sun and not others. Sun sensitivity can develop at any point throughout the course of the illness.

Why does Lupus affect the skin?

As doctors are not sure what causes lupus, the reasons that lupus affects the skin are also unclear. It is known that antibodies and other proteins that are used to fight infection are deposited in the skin inappropriately and cause inflammation. It is also known that proteins within the skin of lupus sufferers are more sensitive to ultraviolet rays contained in sunlight resulting in inflammation.

What treatment is available for the skin in Lupus?

A number of treatments are available for the skin in Lupus. These can be divided into topical, injection and oral treatments. Topical treatments tend to consist of steroid creams and ointments. These can range from the mild creams such as hydrocortisone to the stronger types such as betamethasone. These will sometimes be enough to control mild lupus rashes but should not be used continually, particularly on the face. In discoid lupus particularly troublesome areas can be injected with long acting steroids under the skin to promote healing.

Most people will require oral treatment to control their skin problems. The antimalarials such as chloroquine, hydroxychloroquine and mepacrine are all very useful in controlling skin rashes. They tend to work slowly and need to be taken for a number of months before any effect is seen. Other oral treatments include steroids, which can also be given intravenously if the skin lesions are very severe. Oral and intravenous steroids obviously have a number of side effects and are therefore usually reserved for skin problems that have not responded to topical treatments and the antimalarials. Sometimes skin rashes cannot be controlled with the above treatments or they recur on steroid dose reduction. In these people other drugs such as azathioprine or cyclosporin can be used. These drugs are often given for other problems in lupus such as kidney disease but they can be given for the skin alone in difficult cases.

How can I help myself?

The main way in which lupus sufferers can help themselves is to avoid sun exposure. This means covering up with long sleeves and trousers in the sunlight and wearing a hat if out in the sun for any length of time. The use of UV film on windows may also be necessary for those who are particularly sun-sensitive. Sun block cream, minimum sun protection factor (SPF 15), should be applied to exposed areas of skin, although many patients will require SPF 25 or higher. Sun blocks are available on prescription so ask your doctor for them. These creams need to be applied regularly and repeatedly as they wear off over time. Sunbeds should never be used as they give a concentrated dose of ultraviolet light, which can be very damaging to a lupus sufferer; fake tans are a safer alternative. If a rash develops that does not clear up rapidly then it should be reported to the doctor as soon as possible as a delay in treatment may result in scarring.

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Lupus: the Mouth, Nose and Eyes

How can Lupus affect the mouth and nose?

One of the most common features of lupus is mouth ulcers. This often occurs during flares along with increased joint pains rashes and hair loss. Fortunately in most people when the disease settles the mouth ulcers settle as well, although stress or tiredness can also make mouth ulcers worse. Mouth ulcers can be painful and uncomfortable and it they are an ongoing problem there are simple treatments available such as antiseptic mouthwashes and sometimes steroid lozenges or paste . Occasionally the nose can also be affected in the same way and vaseline cream or steroid paste or nasal spray can be used if necessary. In the vast majority of people these simple measures, and controlling of disease flares with low dose steroids and/or tablets such as hydroxychloroquine, are all that is required. Very occasionally ulceration of the mouth or nose can be more severe and if it does not respond to these simple measures there are more specialist drugs available.

The importance of oral hygiene must not be forgotten. Keeping teeth brushed and giving up smoking are even more important in people with lupus. If the mouth is particularly sore a soft toothbrush can be used.

Can the lupus skin rashes spread inside the mouth?

Some people with lupus can develop discoid skin lesions which are slightly elevated, red areas of skin. The term discoid came from their appearance of looking like red coins or discs on the skin. Similar lesions can occur inside the mouth or nose and occasionally they can also ulcerate.

This type of problem is much less common in people with systemic lupus and is more likely to occur in people whose condition is fairly limited to the skin. Again common sense such as the importance of oral hygiene, stopping smoking etc applies. Depending on the type of lesion, steroid paste and antimalarial tablets such as hydroxychloroquine and/or mepacrine are often used to treat this type of lupus disease. There is some suggestion that people with discoid lupus in the mouth, where it is very florid, may be at greater risk of this turning into cancer. People can read this and get very worried about it. It should be pointed out that this is very unusual and really only applies to the most extreme cases but obviously will be carefully watched for in that very small number of individuals.

Having a chronic condition such as lupus may increase the likelihood slightly of getting some other conditions that can affect the mouth even though they are not necessarily related in their mechanisms. Thus cold sores and oral thrush can sometimes occur. In general the small doses of oral steroids that are used to treat lupus do not mean infections in the mouth are much more likely. However, drugs to prevent thrush will be given where, for example, people are being treated with cyclophosphamide infusions, to try and stop this happening. The lining tissue of the lower bowel and the genital tract is similar to that of the nose and mouth and so often problems that affect one can affect the other.

How can the eyes be affected in lupus?

One of the conditions that can occur along with lupus is Sjögren's syndrome. This occurs in about 1 in 5 to 1 in 10 people with lupus. In Sjögren's syndrome the immune system attacks the glands that produce fluids to lubricate different parts of the body. This most commonly produces dry eyes and dry mouth (xerostomia). It is usually milder in people who also have lupus. Artificial tears or saliva will often help. There are also some special pastilles that can be sucked to help the production of saliva. It is important however that these are sugar-free as the lack of saliva increases the risk of tooth decay. There is a blood test for a specific antibody that is often found in people with Sjögren's (anti-Ro and anti-La antibodies). Sometimes a tiny piece of tissue can be removed form inside the lower lip to detect the condition (a salivary gland lip biopsy). Sjögren's syndrome can also cause dryness of the vagina (which can make sexual intercourse sore), or dryness of the skin. Gels and creams are available to help these symptoms.

At some time about 1 in 10 people with lupus develop conjunctivitis. This usually presents with slightly gritty red eyes which can be sore and itchy. Depending on the cause this may require steroid or antibiotic eyedrops.

Use of long term steroids, particularly at higher doses increases the risk of getting cataracts. It may also increase the likelihood of getting glaucoma. On the whole it must be stressed that this is uncommon, but If there is a family history then regular eye checks by an optician may well be advised. Occasionally people report slight blurring of vision when the steroid dose goes up or down as a result of fluid changes in the eye or when the steroid dose is changed. If you are on hydroxychloroquine (plaquenil) or chloroquine there is a theoretical risk of developing inflammation at the back of the eye. Over recent years however it has become clear that this risk is incredibly small at the doses that we use these drugs at, if indeed there is any risk at all. Nevertheless as a precautionary measure it important to mention any changes in your ability to read or changes in colour vision to your doctor, and from time to time your doctor may recommend an eye check at your opticians. If you have been on this medication for a few years it may be sensible to do this about once a year.

Lupus can, rarely, affect the blood vessels in the eye which may lead to pain and reduced vision. If this happens then you should phone your doctor straight away as it may be important to treat you with steroids and other drugs, whether as drops, or tablets, or injections, quickly to prevent permanent damage occurring. Inflammation inside the eye (iritis or scleritis) can also produce a painful red eye and again urgent treatment may be needed.

Sometimes conditions such as shingles can affect the eye and people who are on steroids or immunosuppressants such as azathioprine or cyclophosphamide or whose spleen is not working well are more prone to infection or reactivation of an infection they have had many years ago. This may require antibiotic or anti-viral treatments. In these circumstances steroids may actually make this type of problem worse. Clearly if there is something very wrong and out of the ordinary, it is important not to waste time but to seek medical advice quickly and get the right treatment straight away.

There is a range of other problems that can affect the eye. Some people with lupus may get an overactive thyroid gland that can occasionally cause prominence of the eyeballs. If there is inflammation of the nerves that supply one or more of the muscles to the eye (optic neuropathy) this may cause double vision when you look in a particular direction or directions. This again is uncommon.

What are the key messages to take home from this?

On the whole lupus affecting the mouth, nose and eyes are generally relatively mild problems. Sjögren's syndrome (dry eyes and dry mouth) occurs frequently but is usually mild. Mouth ulcers are unpleasant but can be dealt with in most cases fairly simply. Similarly mild problems affecting the eyes are common, but if something out of the ordinary develops suddenly, such as decreased vision in one eye, or a red, painful eye, then you may need to see an eye specialist straight away, either through an eye casualty department, if available, or your GP and it may not be sensible to wait until the following day when an appointment is available. In those circumstances it may also be sensible to let your lupus specialist know what is happening so that everybody is aware of what is going on.

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Lupus: fatigue and your lifestyle

What can I do to help with fatigue?

Almost 90% of lupus patients experience fatigue, making it one of the most common symptoms. These symptoms may vary from severe, fleeting or persistent and can form a vicious circle. If you are tired, you don't feel like exercising and if you don't exercise then often you become tired and lethargic.

It is important to ensure a balance between exercise and rest - exercise and rest intermittently as the body allows. Listen to your body, it will tell you when it is time to rest. Increasing stamina allows you to gain more strength and muscle tone. Remember that rest is also needed to restore energy.

What physical activities will I be able to continue with?

Activities that strengthen your stamina and don't cause stress to the joints and muscles are advised. These include swimming to provide an all-over fitness, walking and cycling. Jogging should be avoided as this may prove too strenuous. It is always advisable to ask the advice of your Physiotherapist and or Occupational Therapist when thinking of starting any exercise regime.

What changes will I have to make to my lifestyle?

Remember you are not alone in making changes, there are people who can help you. Everyone is an individual and will cope with their illness in different ways. Lupus itself can alter the way you will cope emotionally. This may also change from day to day. Talking to other people may be an important way to cope. This may be with family and friends, your hospital consultant, nurse other health care professionals. However, you may need to talk to someone who has experienced whet you are going through and this is where by joining LUPUS UK and being a part of a Regional Lupus Group may help.

These groups not only offer a chance to talk to other people with the disease but provide you with an up-to-date awareness of changes and developments in lupus treatment and care. Family members and friends also need help and support to enable them to understand the difficulties you are facing. There is always someone who will spend time with you even if you don't want any answers and all you want is someone to listen to your fears and anxieties.

What about work?

As with exercise, the most important thing is to listen to your own body; it will let you know when you need to take a rest. There is no doubt that some adjustment to your lifestyle will have to be made. However, how you learn to cope with these changes will be an important part of the process of adjustment.

Take time to think of the activities you did before you had lupus. Changes may be necessary but these need not be negative ones. Learning to cope with your lupus and taking control of it and not letting it control you will enable you to act with a positive attitude. It may be that you cannot manage to work the hours you used to work. However, this may not be an option due to financial commitments. There are other ways to overcome this hurdle. You may be able to spread the hours you work over more days or be able to take a break in the day as many continental countries do. It is now more than ever crucial to look after your body, it is no good pushing yourself to the limit. Learn to pamper yourself a little, learn to accept help when offered and don't push yourself to do anything that may be unnecessary - some things can wait until tomorrow.

Remember the body also needs time to rest and rejuvenate and start again.

What changes will my family have to make?

Lupus may initiate the need for changes in your family life; some of these may be permanent, others may be more temporary. There may be some friction between families if for example, you are no longer able to do all the chores you used to be able to do. You may have to ask for and accept more help. Your family may be understandably worried about your health and go to the extreme of not letting you do any of the things you used to do. This will lead to you feeling undervalued and make readjusting your lifestyle harder. Try to think of how this change is affecting your family: they also will need time to adjust. Make sure you find time for your family and try to share your fears and anxieties with them. Consider how you would feel if the change was affecting them. Allow time for the things you enjoyed together before you had lupus and continue to enjoy them.

What about my sex life?

There is no doubt that your appearance and behaviour may change with lupus. You may feel more sensitive to these changes and feel that your partner doesn't understand. It goes without saying that some relationships do end but this is a fact of life and happens even without an illness. You need to be able to communicate what you are feeling, what anxieties you or your partner have and if you can't solve these alone perhaps it is time to seek professional help.

Lupus can certainly affect your sex life and the closeness of your relationship. There may be psychological and physical reasons for your sex life to become affected. Physically you may be experiencing muscle and joint pains and not feel able or want to move around. Pain can restrict the activity of love making, therefore, it is important to try and find ways to relax muscles and reduce pain. Massage, warm baths and relaxation may help. You may need to try different positions to put less strain on joints. Learn to talk to your partner about what is comfortable for both of you. Take into consideration the time of day you make love. If you experience early morning loss of mobility you might want to restrict your love making to afternoons or evenings. Fatigue as previously mentioned can affect 90% of Lupus sufferers. Therefore, if you are tired you will not enjoy lovemaking. However, talk to your partner and let them know why you don't want to make love. Being close to someone in their arms can also be a loving experience. Other problems that may be encountered are:

Drugs may be painkillers that make you feel drowsy. As with coping with the pain, find time when you have adequate pain relief without feeling drowsy. Some drugs, eg beta blockers, can diminish sex drive. It may be possible, on advice from your doctor, to change the brand of drug being used to one which does not have this effect. If this is not possible you should explain to your partner that your medication is responsible for your lack of libido and that you are not just 'cooling off' your relationship! With Reynauds, the circulation of blood being restricted may also cause you to feel pain and uncomfortable during love making. Warm baths and a warm atmosphere can help.

Dryness - just as some people develop sore eyes and mouth you can also develop a sore and even sometimes ulcerated vagina. Ulcers should not necessarily inhibit lovemaking. Soreness and dryness may be overcome with longer foreplay to increase lubrication or by using lubricants such as KY jelly. If this soreness and dryness persist it may be necessary to seek medical advice. Don't be embarrassed, you will not be the first person to consult your doctor about this problem.

Social life and friendships

Your friends will react to your condition in a similar way to your family and it is up to you to decide how much or how little you think they need to know. As for your social life, let your body lead you in this. Do as much as your body allows and continue to enjoy your life like many others with lupus do.

Will lupus affect my finances?

It you find you are unable to work for long periods of time it may be there are benefits you can claim. A social worker should be able to help you with any enquires you have. You will usually need to give the name of someone who knows about you and your condition when completing forms. This is often your lupus specialist, nurse or GP. If you don't ask you won't know if you are entitled to financial help.

Hopefully this fact sheet has helped answer some of the questions you have regarding changes to your lifestyle and how to cope with fatigue, but it is important to remember you never have to be on your own - there is always someone to listen to your fears and anxieties. Your family and friends, your lupus specialist or nurse and LUPUS UK and local lupus group contacts are just a few of the important people who will help you come to terms with your illness.

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Lupus and Men

Although systemic lupus erythematosus (SLE or lupus for short) is about ten times more common in women than it is in men, it is important not to forget the men with lupus and the effects of the disease on their health and life. In particular, the development of a chronic disease may necessitate life style changes which can alter their traditional role within the family.

Hormonal influences on lupus

The role of sex hormones in lupus is suggested by observations showing that about ten times as many women as men have lupus. The evidence suggests that hormones can exert an important influence en the immune system and, in particular, that the female hormone oestrogen can affect the development of lupus. Some studies have suggested that there are differences in the way that sex hormones are chemically changed in lupus patients. Males and females with lupus have increased amounts of 16-hydroxylated oestrogens, and female, but not male, lupus patients have increased oestriol. The 16-hydroxylated compounds are the ones which can interact with cells of the immune system and which may be important in lupus. Levels of these compounds can be increased by talking the oral contraceptive pills containing oestrogen. These contraceptive pills have been reported to trigger the start of lupus or to cause it to have greater impact on some female patients.

Some studies have suggested that levels of testosterone, one of the male hormones or androgens, are reduced in males with lupus but these observations have not been supported in the majority of studies. However, there is a difference in the way that the male hormone androgen is metabolised (processed) between the sexes. There is a suggestion that women with lupus metabolise androgens at a faster rate than those without lupus and, consequently, have lower levels of certain androgens. Androgens have been shown to have some immunosuppressive properties. Recently, studies have been started in which androgens are being tested as a treatment for lupus in women, but the potential value of such treatment for males with normal androgen levels is unclear. For the few men with low androgen levels they may be of benefit, and would reduce the risks of osteoporosis, even if they are not effective at suppressing lupus disease.

Clinical disease in men

Although lupus is uncommon in males, it does not differ clinically in major ways from the presentation of lupus in females. Some have suggested that lupus is more severe in males but most studies have not found this to be so. The difference may be explained by a delay in diagnosing or treating lupus in males, as the disease is less often thought of in men, being more common in females, and because men may be more reluctant to seek medical advice than women.

The aspects of lupus which have been reported to be increased in males in some studies include skin rashes, pleurisy, neurological disease affecting the nerves in the arms and legs (peripheral neuropathy), kidney disease, vasculitis and Raynauds phenomenon. Impotence has been reported in some patients with abnormal hormones, particularly low androgen levels. More men develop drug-induced lupus than women mainly because medications that produce drug induced lupus are more frequently used in men. These drugs include hydralazine and procainamide, and may reflect more heart disease in men than women. However, in recent studies it has been shown that it is women with lupus, particularly those in their forties, who are at increased risk of heart attack and stroke compared with women without lupus of the same age and whether men with lupus are at greater risk of these conditions than men without lupus of the same age is not yet clear.

Lifestyle considerations

Men may react differently from women when the diagnosis of lupus is given. This may be because of the misconception that lupus is a woman's disease giving rise to the idea that a man with lupus is less masculine than his colleagues without lupus. This is not true. As discussed above, most men with lupus, as far as sex hormones and sexual function are concerned, are no different from males without lupus. Men with lupus are usually sexually active, potent and have normal reproductive histories. There may be some interruption in this when the disease is very active or with the use of certain drugs, especially cyclophosphamide which can affect fertility (see Lupus and Medication fact sheet).

Many of the day to day stresses in life are equal emotionally for men and women with lupus. However, in some ways it may be more difficult for men to cope with having lupus because of the pressures created by what they think their family, friends and society expect of them. They may not be able to work or follow hobbies in the environment previously expected of them (eg in the cold due to Raynaud's phenomenon). There may be difficulties in performing activities that require physical strength or prolonged periods of physical labour and a change of role in the work place may be necessary but not available. Historically it has been the male who is the provider for the family. If a man with lupus has to stop working or change job this may result in significant financial and emotional stress. However, this is by no means necessary in most men (or women) with lupus. With earlier diagnosis and better treatment, many patients can remain in work, although some will have to change job, modify duties, or reduce the hours compared with their colleagues. Combined advice from an occupational health physician at the workplace and the lupus physician may be required to establish what is appropriate work for an individual with lupus. Similarly, lupus patients may have to discuss with their family and consultant how to adjust their leisure activities and hobbies, as it is essential that they get enough rest and remain as physically and emotionally fit as possible.

Another aspect of the disease that men (and women) have to cope with is the change in physical appearance. Rashes, unexpected hair loss and weight loss or gain may not be appreciated as being as important to men as to women However, they may result in further loss of self esteem and the feeling of loss of masculinity, particularly in young men. More serious problems such as disability due to stroke causing paralysis of one side of the body is even harder to cope with and may cause profound depression which needs active treatment as well as physical rehabilitation by physiotherapists.

Often men find it difficult to talk about health matters or seek support from self-help groups. They can appreciate one to one informal chats more than taking part in predominantly female meetings.

Conclusion

Men, like women, will need to discuss their lupus disease and its likely effects on their health and lifestyle with their consultant. Every case is different but certain generalisations will be possible based on the clinical picture (what parts of the body are affected) and the results of blood tests and other investigations. There is no definite evidence that the disease is more severe in men than women, or that men with lupus have more female hormones or less male hormones than those without lupus. Fertility is not usually affected but active disease and certain drugs may affect sexual function and this needs to be discussed openly by the patient and his physician.

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Lupus and light sensitivity

Systemic lupus erythematosus (SLE or lupus) is a multisystem disease with a tendency to cause skin rashes. These often appear on light exposed skin after exposure to sunlight (for example face, neck, hands, and feet). These rashes are called photosensitive rashes and are one of the hallmarks of the disease (see relevant lupus fact sheet). Some patients complain of feeling unwell after going out in the sun, even for relatively short periods of time (such as half an hour). For example, they may develop migraine, nausea (feeling sick) or joint pains. The joints may even become tender to touch and swollen. A true attack of arthritis due to increased activity of the disease (a flare) may follow. These are all manifestations of light sensitivity in lupus patients. Other aspects of the disease may deteriorate after sun exposure, including fever, pleurisy (chest pains on breathing in), kidney disease and more serious nervous system problems such as epilepsy (fits). Occasionally, patients are sensitive to fluorescent lighting but this is much less common than sun sensitivity.

What is it in sunlight that causes lupus to flare and causes rashes to develop in particular?

It is the ultraviolet (UV) waves in the sunlight. Ultraviolet light in the UVA and UVB wavebands is responsible. UV light damages cells in the skin (keratinocytes) causing them to die. In healthy people without lupus, these dead cells are cleared away quickly and any inflammation induced by the sun-induced skin damage is short-lived (sunburn).

However in lupus patients, the skin cells may be more sensitive to sun-induced damage and there is increasing evidence that the dying (apoptotic) cells are not cleared away efficiently. As a result the contents of the dying cells may be released and cause inflammation. Also, cell contents such as DNA (the genetic material) and other molecules including Ro which are never normally exposed to cells of the immune system are available to generate (start) an immune response. Immune responses and inflammation are the normal reactions of the body to infection, but here they are being generated inappropriately by the dying cells and the body mounts an immune response against its own cell constituents (autoimmunity). The end result of this process in susceptible people with certain types of lupus is the development of characteristic photosensitive rashes. Antibodies to Ro, in particular, are often found in people with these rashes. Why the rashes affect only some parts of the body at any one time and are not always sun-sensitive is still not understood.

Why do some people with lupus get other disease manifestations after sun exposure?

The immune response to cell constituents results in the formation of these special proteins called antibodies. The antibodies are made by white blood cells called lymphocytes that circulate in the blood and both the lymphocytes and the antibodies can travel to different parts of the body.

These antibodies in lupus are directed against, and bind to, particular molecules such as DNA and Ro. These cell constituents may be released by other dying cells in the body as it appears that the inability to clear dying cells is not limited to the skin. Wherever the antibodies and their target molecules meet up and bind together, an immune complex is formed which can set up a series of inflammatory processes causing disease manifestations in that part of the body. Having said this, it is still not entirely clear why different people with lupus get certain disease manifestations and not others.

Do all lupus patients suffer from light sensitivity?

No, about 60% of lupus patients get sun-induced rashes and a further 10-20% complain of other clearly sun-induced problems. The role of sunlight in the remainder is unclear. Only a few people are confident that sun exposure definitely does not affect them, as they can go out for long periods and sunbathe without any ill-effects then or in the following weeks. Because new immune responses can take over a week to develop, the effects of sunlight will not necessarily be on the same day. In general, all lupus patients are advised to avoid sun exposure as it is one of the easiest ways of avoiding something which we know can make lupus worse in many sufferers.

Can photosensitive rashes and other sun-induced manifestations of lupus be prevented?

Yes, to a large extent photosensitive rashes and other sun-induced problems can be prevented by keeping sun exposure to a minimum and using sunblock regularly in the summer months (often from April to October). This also means not going to hot sunny countries or mountainous areas where there is more UV light than in the UK. In particular, beware the increased UV exposure with snow, sea or other water due to additional light reflected on to the body (especially the neck and chin!). Even in the UK, it is wise to avoid going out in the sun in the middle of the day in summer. Sunblock should be sun protection factor (SPF) 25 or greater and effective against UVA and UVB light. It should be put on in the morning and reapplied during the day (at least once or twice) as it tends to get rubbed off or sweated away, particularly in warm weather, and don't forget your hands and feet! Sunblock should be used even on cloudy days by light-sensitive people because UV light can penetrate the cloud layer and you can never tell what the weather will be like later on. It is also advisable to cover up with long sleeves and trousers and wear a wide-brimmed hat when out in the sun. The use of UV film on windows may also be necessary for those who are particularly sun-sensitive.

How can I afford to buy so much sunblock?

Certain types of sunblock are available on prescription for people with a sun-induced disease like lupus. The general practitioner (GP) usually prescribes the sunblock but the GP may want advice from a hospital specialist about which one to prescribe, how often and for how long. If you prefer a particular sunblock that is not available on prescription then you will have to pay for it yourself.

Do drugs provide protection against sun-induced flares of disease?

Yes, some drugs do help by damping down the immune responses and inflammatory processes. Steroids (for example prednisolone) will help to prevent and treat lupus manifestations due to sunlight. However, it is always best to be on the smallest possible dose of steroids, so avoiding UV light and wearing sunblock is important even if you are on steroids. Hydroxychloroquine (Plaquenil) seems to be particularly helpful at preventing rashes, arthritis and pleurisy which may be sun-induced, but is not a replacement for sensible behaviour. Other drugs (such as azathioprine, methotrexate, cyclophosphamide) which are often used for more serious disease or to keep the dose of steroids as low as possible may also reduce the risks of sun-induced flares.

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Lupus and pregnancy

Is pregnancy possible in lupus? The answer is very definitely yes. As recently as ten years ago many patients were routinely advised against pregnancy in lupus but this is now known to be incorrect. However, it is advisable for a woman with lupus and her partner to discuss with an obstetrician all the issues likely to be involved in her particular case before embarking on a pregnancy.

What are the problems?

There are two major potential problems. Firstly, an increased risk of miscarriage in those patients with anticardiolipin antibodies (see fact sheet on antiphospholipid (Hughes) syndrome). Secondly, a chance of a flare of lupus after the baby is born - in the 'puerperium'.

Does the lupus get worse in pregnancy?

In the majority of cases it does not although flares occur with almost the same frequency as in the non-pregnant state.

What if I have kidney disease?

Where there is kidney disease the success of pregnancy clearly depends on the degree of kidney involvement. Blood pressure control is far more difficult where kidney disease is present but it is now recognised that lupus patients with definite mild kidney disease can have successful pregnancies.

Recurrent miscarriages

Lupus patients should all be tested for antiphospholipid antibodies (see antiphospholipid (Hughes) syndrome). If these tests are positive there is an increased risk of miscarriage and patients are treated accordingly - either with aspirin or heparin during the pregnancy. In those patients with lupus who are anticardiolipin negative it is probably true to say that there is not an increased risk of spontaneous miscarriage.

Drugs and pregnancy

Drugs which are contraindicated in pregnancy include warfarin, which can affect the foetus in the early development stage, cyclophosphamide and methotrexate. Low dose antimalarials (plaquenil 200mg daily for example) appear to be safe in pregnancy and can continue to be used by those patients already on this drug. It may come as a surprise to some that the safest medicine of all in pregnancy is steroid (a 'hormone') and flares during pregnancy are routinely treated by changing the steroid dosage.

Will my baby be fine?

Lupus mothers have normal babies. However, there is one congenital problem which has been described in rare cases. Approximately 1 in 1000 babies of lupus mothers are born with a slow pulse rate, so-called congenital heart block. This congenital abnormality is only seen in offspring of mothers with a particular antibody anti-Ro (see fact sheet on Lupus and Laboratory Tests).

The anti-Ro antibody may also, very rarely, give rise to a lupus-like rash which will usually disappear in the first twelve months.

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Lupus and blood disorders

Systemic lupus erythematosus (SLE or Lupus) has many clinical and laboratory manifestations. This article relates to those manifestations which concern the cells and clotting factors that circulate in the blood. As with other manifestations of lupus, the underlying cause is usually the presence of antibodies, which are proteins that recognise and bind to other proteins in the body However, instead of getting rid of an unwanted foreign protein (which is what normal antibodies do), the antibodies in lupus recognise self-molecules (usually proteins) and interfere with the normal function of our own proteins and the cells containing the proteins.

What is blood?

Blood is made up of cells and a protein rich liquid containing many substances including antibodies (serum). The cellular part is composed of red cells (which contain the oxygen carrying molecule haemoglobin), white cells (which fight infection and can be subdivided into several types, of which the neutrophils and lymphocytes are particularly important) and platelets (which are involved in dotting the process which stops us bleeding when we cut ourselves). Clotting also involves a number of special proteins called clotting factors in addition to the platelets. A full blood count test measures the number of red and white cells, platelets and the amount of haemoglobin circulating in the blood. There are special tests for assessing the clotting properties of blood (see below under 'how is antiphospholipid syndrome diagnosed?')

Why do lupus patients have low white cell counts, and what is the significance of this?

In SLE, antibodies directed against white cells are very common. A lower than normal lymphocyte count is found on the full blood count in about 95% of lupus patients. This is due to the presence of antibodies to lymphocytes which results in the destruction of the antibody-coated lymphocytes. Fortunately this rarely causes a clinical problem because more lymphocytes are released from the bone marrow where they are made. This means that there are enough lymphocytes to fight infection, especially those due to viruses like influenza ('flu'). However, high doses of certain drugs can also cause destruction of lymphocytes and, because the bone marrow where these cells are made is also attacked by such drugs, there may not be enough lymphocytes to light infection. So in this case there is an increased risk of infections, especially viral infections. Cyclophosphamide, which is sometimes used to treat more severe forms of lupus such as kidney disease, can have this effect. Consequently the white cell count is regularly checked in people receiving this drug so that the drug dose can be adjusted if necessary.

Low neutrophil counts are less common than low lymphocytes counts as a result of lupus and is more common in people of Afro-Caribbean origin (even in the absence of lupus). Low neutrophil counts can also be the result of drugs such as cyclophosphamide and azathioprine and if severe can be associated with bacterial infections such as pneumococcal pneumonia. Again, regular blood monitoring tests will usually prevent this complication by allowing the drug dose to be adjusted before a problem occurs. Why do lupus patients become anaemic and what effect does this have? Anaemia means that there are less red cells (and therefore less haemoglobin to carry oxygen) in the blood than there should he. Low red cell counts and the associated low level of haemoglobin in the blood can result from the effect of antibodies attacking the red cells and causing their destruction, a process called haemolytic anaemia. It is more common though to have anaemia due to poor production of red cells in the hone marrow. This usually occurs as a side-effect of general inflammation in the body due to lupus. Rarely is anaemia caused by drugs but this is possible. Whatever the underlying cause of anaemia, the end result is to cause the person to feel tired (fatigue) in proportion to the loss of red cells (haemoglobin), although there are other (often less well understood) causes of fatigue in lupus. In more severe cases, the person may become short of breath even in the absence of lung disease because there is not enough oxygen in the blood.

What are the effects and causes of low platelets in lupus patients?

Low platelets are usually due to antibodies and less commonly the result of drug side-effects. When the count is very low there is an increased risk of bruising and bleeding; fortunately this is a rare manifestation of lupus. Surprisingly there is another type of antibody that can reduce the platelet count (usually only mildly), so there is no bleeding but instead these antibodies can interfere with platelet function and this causes increased clotting, known as thrombosis. This is a painful condition, as not enough blood can get through a blood vessel containing a clot and this causes damage to that part of the body. This can occur, for example, in the calf (deep vein thrombosis), and the clot can spread from a leg vein through the circulation to the lungs (pulmonary embolism). The underlying cause of this thrombotic condition is known as antiphospholipid syndrome, as there is a group of antibodies present which interferes with phospholipids in platelets and other cells and proteins involved in clotting.

What else does the antiphospholipid syndrome cause?

Apart from causing low platelet counts, deep vein thrombosis and possibly pulmonary embolism, antiphospholipid antibodies can cause blood clots in the major arteries of the body This can result in conditions such as heart attack or stroke. In women of childbearing age, these antibodies can also cause miscarriages or stillbirths due to blood clots forming in the placenta which prevent nutrients from reaching the developing baby during pregnancy.

How is antiphospholipid syndrome caused?

Antiphospholipid syndrome is diagnosed by finding antiphospholipid antibodies in the blood and clinical features of increased blood clotting (for example recurrent deep vein thromboses or miscarriages), The antibodies can be detected by a specific test for the type of antibody, such as anticardiolipin antibodies, or by abnormalities In conventional clotting tests. Antiphospholipid antibodies interfere with many of the conventional clotting tests because these tests depend on phospholipid for the test to work normally. If antiphospholipid antibodies are present, they prevent the blood from clotting, making it look as if there is a shortage of clotting factors, However, in reality the antibody acts as an inhibitor of the clotting test and in real life this is associated with an increased risk of clotting (this paradox is confusing to everyone). If you mix the serum part of the blood containing the antibodies with serum from a healthy person, the antiphospholipid antibodies still interfere with the clotting test, whereas if there is a clotting factor deficiency (as in haemophilia), the mixing results in correction of the missing factor and the clotting test becomes normal. This is the basis for some special tests of blood clotting used to diagnose antiphospholipid syndrome.

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Lupus and Laboratory tests

One of the most typical features of lupus is the presence of particular antibodies in the blood. Antibodies are proteins which recognise and bind to other molecules (usually proteins) in the body. They are usually produced in response to infection. However, instead of getting rid of an unwanted foreign protein (which is what normal antibodies do), the antibodies in lupus recognise components of our own cells (usually DNA or proteins). These antibodies are therefore called auto-antibodies. Why these auto-antibodies are made in lupus is complex and still not fully understood. Binding of the auto-antibody to its target can interfere with the normal function of the target molecules, the cells containing the molecules, or can result in the formation of complexes containing the antibody and its target molecule (called immune complexes) which become trapped in blood vessels and the kidney and cause inflammation and damage. This damage is often due to the activation of a series of proteins called complement (also normally involved in clearing infections from the body). Thus laboratory tests in lupus are performed in order to assess the activity of the disease (for example The type and amount of autoantibodies and complement in the blood), and the effects of the disease and certain drugs used to treat lupus on blood cell counts and blood chemistry.

What is an ANA test?

ANA stands for anti-nuclear antibody. This test detects a group of antibodies directed against components of nucleus of the cell, such as DNA and ribonucleoproteins (RNP). The individual antibodies include anti-DNA antibodies and the various anti-ENA antibodies (see below). The ANA test is used as a screening test for these autoantibodies which may then be identified individually by other tests. The ANA test is positive in 95% of people with lupus but only about 5% of healthy people. It can also be positive in people with related autoimmune conditions (sometimes called connective tissue diseases) such as dermatomyositis, polymyositis, and systemic sclerosis (scleroderma). It is sometimes positive in people with other types of disease such as chronic infection or certain malignancies (cancers). It is therefore not diagnostic of lupus, but it is important supporting evidence when other features (symptoms, signs and other laboratory tests) suggest lupus.

Why is the ANA test done to diagnose lupus?

The ANA test is done as a screening test as it is the commonest laboratory abnormality found in lupus. However, for the diagnosis of lupus to be confirmed it must be associated with several other clinical and/or laboratory features of lupus and no other conditions that can cause a positive ANA. Appropriate clinical features include one of the typical skin rashes, recurrent mouth ulcers, inflammatory arthritis, pleurisy, and epileptic fits, but there are many other possible manifestations. So in anyone suspected of lupus on clinical grounds (symptoms and signs on examination), the ANA test is one of the simplest tests to help establish the diagnosis, as all these clinical problems can result from other conditions. But it must be remembered that certain other conditions can cause a positive ANA test and some healthy people have these antibodies (particularly at low levels in the blood). This explains why it can be hard to diagnose and distinguish lupus from other conditions in the early stages. The diagnosis of lupus becomes more certain as more clinical and laboratory features are found with time.

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Lupus and Medication

The types of drugs used in lupus can be broadly divided into those which treat the disease itself (e.g. antimalarials) and those used for other problems sometimes associated with lupus (e.g. blood pressure tablets)

Non-steroidals

These are the standard drugs used for rheumatic pains. There are many to choose between and they have a good safety profile. They are designed to lower inflammation and are, therefore, widely used in all the rheumatic diseases. The main drawback is that they are prone to cause indigestion, especially in older patients.

Anti-malarials

The drug hydroxychloroquine (Plaquenil) is widely used for lupus. It has a number of properties which make it potentially useful, having anti-inflammatory properties, some sun-protective properties and the property of giving some protection against clotting. The older drug chloroquine used to be more widely used but there is a suspicion that higher doses could cause eye (retinal) damage. Recent studies with hydroxychloroquine at a dose of one tablet (200mg) daily show that the risk of retinal disease is minimal. Antimalarials are particularly useful for skin rashes, for joint pains and for helping the fatigue of lupus.

Corticosteroids (prednisolone)

These are life-saving in acute lupus and have totally changed the outcome of the disease. Modern treatment is geared to reduce the dose as much as possible and it is now known that the majority of lupus patients can be either maintained on a low dose or even weaned off the drugs altogether. The side-effects of steroids are well known and include weight gain and "moon" face, muscle weakness and, over a period of time, bone softening or osteoporosis.

Immunosupressives

The two most widely-used drugs are azathioprine and cyclophosphamide. Azathioprine is a milder immunosuppressive and is used for mild to moderate kidney disease or where it is proving difficult to reduce the steroid dosage. Cyclophosphamide, now always given if possible as an injection or "pulse", is widely used for kidney disease and to a lesser extent for severe neuropsychiatric disease, It is a very effective drug and the newer regimes using lower doses by injection have a much higher safety profile. Major side-effects of cyclophosphamide are a reduction in white cell count and, with the use of higher doses, failure of the ovaries or sperm-producing cells in males.

Other drugs

Other drugs are less frequently used in lupus and include intravenous immunoglobulin (often used when the platelets are low) and cyclosporin A, the drug widely used in transplantation medicine to suppress rejection, For very severe skin disease in patients where pregnancy is not a consideration, thalidomide has proved an extremely powerful medication.

Non-lupus drugs

Various medications have helped improve the prognosis in lupus. These include a variety of improved blood pressure tablets and diuretics, anticoagulants (aspirin or warfarin) in those patients with a clotting tendency, anti-epileptic and anti depressive medication. Skin creams include corticosteroids and newer, vastly improved sun-protection creams. Patients who have received long-term steroids are a increased risk from osteoporosis, There are now, in addition to standard calcium and vitamin D preparations, modern effective drugs for the prevention and treatment of osteoporosis.

Finally, HRT (hormone replacement therapy and the Pill. The majority of young women with lupus suffer no problems on the oral contraceptive pill. However, it is now recognised that those women with antiphospholipid antibodies are at increased risk of thrombosis or migraine when taking the pill. Perhaps surprisingly, hormone replacement therapy, often given to treat osteoporosis in post-menopausal lupus patients, is generally well tolerated with few side-effects.

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Lupus and Mixed Connective Tissue Disease

What parts of the body does lupus usually affect?

Systemic lupus erythematosus (SLE or lupus) is one of the so-called connective tissue disorders that is caused by a fault in the body's immune system which normally fights infection. In this condition, antibodies are made, which instead of killing germs, attack the body itself and cause damage.

Lupus can vary from a very mild disease for which no treatment is required to a much more serious condition which needs very strong medication. Most people lie somewhere between these extremes. A rash is a very common problem often made worse by the sun (photosensitivity). Mild hair loss (which comes and goes) can be a feature of the disease. Joint pains, particularly in the hands and feet, can also be a problem as can general tiredness. Sometimes the circulation into the fingers can be affected and this causes them to go white and numb in cold weather. As the fingers recover they turn blue then red. This is called Raynaud's. In some people with lupus the blood clots more easily than it should do.

Lupus can affect many other parts of the body including the kidneys, brain, nerves and lungs. This is much less common. However, when lupus does this it often requires very powerful medication. Kidney problems can also cause high blood pressure.

Are people with lupus more likely to get other problems where the immune system attacks the body?

The answer to this is yes. Anyone with one condition where the immune defence mechanism attacks the body (so-called autoimmunity) is prone to another one. We know this is the situation with all autoimmune diseases. For example, people with diabetes (where the body attacks its insulin making cells) are more likely than the general population to get rheumatoid arthritis or thyroid diseases.

In general, if a patient has lupus combined with another autoimmune disease, the lupus itself tends to be less severe. In the same way, the other autoimmune disease, which can occur without lupus, tends to be milder. Correspondingly, doctors can get away with less powerful treatment in these 'overlap' or 'mixed connective tissue' disorders.

What are the other problems that people with lupus are prone to?

One of the conditions that can occur along with lupus is Sjögren's syndrome. This can occur in about 1 in 5 patients with lupus. With this problem the immune system attacks the glands that produce fluids to lubricate different parts of the body. Therefore dry eyes and dry mouth can be a problem. The doctor can provide artificial tears or saliva to help with this. There are some special fruit pastilles that can be sucked to help the production of saliva. However, it is important that these are sugar-free as the lack of saliva increases the risk of tooth decay. There is a blood test for a specific antibody that goes with Sjögren's syndrome. Sometimes a tiny piece of tissue can be removed from the lower lip to detect the condition (a lip salivary gland biopsy).

Sjögren's syndrome can also cause dryness of the vagina. This can make sexual intercourse sore. There are lubricants available to help with this too.

A little under 1 in 10 patients with lupus suffer with another problem of autoimmunity. This is where the body attacks the thyroid gland that controls the body's metabolism. The thyroid gland can either become underactive or overactive. If the gland is overactive, it can cause sweating, anxiety, shaking, heart pounding and weight loss, if underactive the skin becomes dry, weight gain is a problem and you can slow down mentally. Sometimes the thyroid gland (which is found at the front of the neck) can become swollen. A thyroid problem is easily detected with a simple blood test. It is also quite easily treated.

In lupus, as discussed above, joint pain is a common problem but this pain is not associated with actual damage to the joint itself. Much less often, people with lupus can develop an arthritis where the joints are affected in the same way as is found in rheumatoid arthritis. Therefore lupus and rheumatoid arthritis can happen in the same person. In rheumatoid arthritis, there is swelling of the lining of the joints. This swollen lining is called the synovium. Normally it is very thin and stops the natural fluid lubrication of the joint from leaking out. However, when inflamed it becomes very swollen, red and angry. It can eat its way into the bones and cause damage to them. Doctors use tablets in this situation to reduce the swelling of the synovium. By doing this they hope to reduce or halt the damage that can happen. The choice of tablets for this when lupus overlaps with rheumatoid arthritis are very similar to those used in rheumatoid arthritis alone.

The muscles of the body can also become inflamed in lupus. Again this can happen in patients without lupus and the treatments are very similar. The muscles can become very painful and weak if this is a problem. There are special blood tests and muscle electrical tests used to look for damage to the muscles from inflammation. Sometimes a small piece of muscle needs to be removed to look for the problem under the microscope (muscle biopsy).

Another problem with the immune system that can occur with lupus is thickening of the skin which causes it to become light and hard -particularly over the fingers and face. This is called scleroderma and is very rare. People with this condition are very prone to Raynaud's. It can also cause the thickening of other tissues which can cause difficulty in swallowing and diarrhoea. There are simple treatments to help with the Raynauds, swallowing and diarrhoea but skin thickening is very difficult to treat and tends to be permanent. Patients with scleroderma without lupus are prone to scarring of the lung and involvement of the kidneys in the same way as is found in lupus itself.

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Lupus and Associated Conditions

Hughes' syndrome (antiphospholipid syndrome)

This is often called "sticky blood" in that the patients have a tendency to clots both in the veins and arteries. The thrombosis may present either dramatically or over a period of time with clots in major vessels, even including the brain. It is vital that this diagnosis is made (a simple blood test for antiphospholipid antibodies is available in most major hospitals). Pregnant women who have antiphospholipid antibodies have a tendency to clot the placenta and develop recurrent miscarriages and these can be prevented by diagnosis and treatment. Although this syndrome was first discovered in lupus patients it is now recognised that many, if not the majority, of patients with Hughes' syndrome have no other features of lupus nor will they develop lupu