Lupus

Introduction
Lupus is a complex and poorly understood condition that affects many parts of the body and causes symptoms ranging from mild to life-threatening. Some common symptoms of lupus include:
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fatigue skin rash joint pain and swelling

Autoimmune condition
Lupus is an autoimmune condition, which means it is caused by problems with the immune system (the bodys natural defence against illness and infection). In people with lupus, for reasons not clearly understood, the immune system starts to attack healthy cells, tissue and organs. As with other more common autoimmune conditions, such as rheumatoid arthritis, it is thought a combination of genetic and environmental factors is responsible for triggering lupus in certain people. Read more information about the causes of lupus.

Types of lupus
There are several types of lupus. The main types are:
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discoid lupus erythematosus drug-induced lupus systemic lupus erythematosus

These are briefly outlined below.

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Discoid lupus erythematosus Discoid lupus erythematosus (DLE) is a mild form of lupus that only affects the skin. It causes symptoms such as:
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red, circular, scaly marks on the skin that can thicken and scar hair loss permanent bald patches

DLE can usually be successfully controlled using medication and by avoiding exposure to direct sunlight. DLE usually only affects the skin but, in some cases, it can progress to the bodys tissues and organs. Drug-induced lupus There are over 100 medications known to cause lupus-like side effects in certain people. Lupus caused by medication is known as drug-induced lupus. Stopping the course of medication will usually help resolve symptoms of drug-induced lupus. However, do not suddenly stop taking any prescribed medication without first checking with your GP. Systemic lupus erythematosus Systemic lupus erythematosus (SLE) is a type of lupus that can affect any body tissue and organ. SLE is what most people mean when they use the term 'lupus'. The symptoms of SLE can range from mild to severe. Many people will experience long periods of time with few or no symptoms before experiencing a sudden flare-up where their symptoms are particularly severe. However, even mild cases of SLE can have a considerable impact on a persons quality of life because many of the symptoms, particularly pain and chronic fatigue, can be distressing and cause feelings of depression and anxiety. The rest of this article will focus on SLE.

Who is affected?
SLE is an uncommon condition, with 90% of cases occurring in women. Most cases of SLE first begin in women who are of childbearing age (between the ages of 15 and 50).

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There are marked differences in how widespread SLE is among different ethnic groups. For example, in the UK:
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one in every 5,000 white women will develop SLE one in every 1,000 women of Chinese origin will develop SLE one in every 625 African Caribbean women will develop SLE

The condition can be difficult to diagnose as there are many other common conditions with similar symptoms. Read more about how lupus is diagnosed.

Treating lupus
There is currently no cure for SLE, but there are different medications that can help relieve many of the symptoms and prevent progressive organ damage. With good levels of support from friends, family and healthcare professionals, many people with SLE learn to manage their condition effectively. 50 years ago SLE was regarded as a terminal condition, as most people would die of a complication in the first 10 years after being diagnosed. Thanks to advances in treatments, now most people with SLE have a normal, or near normal, life expectancy. Read more information about how lupus is treated.

Complications of lupus
SLE can sometimes cause serious complications, such as kidney failure or heart disease, and the treatment may make a person vulnerable to serious infections. Also, around one in three people with SLE have another autoimmune condition, such as thyroid disease, Sjogrens syndrome or Hughes syndrome (antiphospholipid syndrome). In some cases this can lead to complications, including problems during pregnancy. Read more information about the complications of lupus.

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Lupus is caused by a fault in the immune system.

Symptoms of lupus
Symptoms of systemic lupus erythematosus (SLE) can vary from person to person. Some people with the condition may only experience a few mild symptoms, whereas others may be more severely affected. The symptoms of SLE can also appear in 'flare-ups'. This means although you may always have mild symptoms, during a flare-up your symptoms may become more severe or new features may appear.

Primary symptoms
The three primary symptoms of SLE are:
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fatigue joint pain skin rashes

These are described in more detail below. Fatigue

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Fatigue is one of the most common symptoms of SLE. You may feel very tired even though you get plenty of sleep. Carrying out everyday tasks, such as household chores or office work, can leave you feeling exhausted. Many people with SLE have reported that fatigue is the most distressing and disruptive aspect of SLE because it has a negative impact on their work and social life. Joint pain If you have SLE, you are most likely to experience joint pain in your hands and feet. You may find the pain changes from one set of joints to another quite quickly. However, unlike some other conditions that affect the joints, SLE is unlikely to cause your joints to become permanently damaged or deformed. Skin rashes In people with SLE, skin rashes most commonly develop on the face, wrists and hands. A rash over the cheeks and the bridge of the nose is particularly common and is known as a 'butterfly rash'. Skin rashes caused by SLE may be permanent and can be made worse by exposure to sunlight. This is known as photosensitivity.

Other symptoms
As well as the primary symptoms listed above, SLE can also cause other symptoms. However, it is unlikely you will have all of the symptoms listed below, and many people with the condition will only experience primary symptoms. Other features of SLE may include:
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high temperature (fever) of 38C (100.4F) or above swollen lymph glands (small, bean-shaped glands found throughout your body, including your neck, armpits and groin) recurring mouth ulcers hair loss (alopecia) high blood pressure (hypertension) depression dry eyes memory loss headaches and migraine shortness of breath due to lung inflammation, heart involvement or anaemia a condition where a lack of

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red, oxygen-carrying blood cells can cause fatigue and shortness of breath Raynaud's phenomenon a condition that limits the blood supply to your hands and feet when it is cold ankle swelling and fluid retention (oedema)

Causes of lupus
Systemic lupus erythematosus (SLE) is an autoimmune condition, which means it is caused by problems with the immune system. The immune system is the bodys natural defence against illness and infection. When the immune system detects the presence of an infectious agent, such as a virus or bacteria, it sends specialist proteins called antibodies to kill it. However, in cases of SLE, antibodies released by the immune system attack healthy tissue, cells and organs. Most experts think SLE has more than one cause. They argue there may be a number of genetic factors that make people more susceptible. However, it also takes one or more environmental factors to trigger SLE in those who are genetically susceptible. Possible genetic and environmental factors are discussed in more detail below.

Genetic factors
It is believed genes you inherit from your parents make you more susceptible to SLE because evidence shows:
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If you have an identical twin with SLE you have an estimated one-in-four chance of developing the condition yourself. Rates of SLE vary widely among different ethnic groups.

Researchers have identified a number of different genetic mutations that seem to make people more susceptible to developing SLE. A genetic mutation occurs when normal instructions carried in certain genes become 'scrambled', resulting in the bodys processes not working normally.

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Most mutated genes are associated with regulating certain functions of the immune system, which may explain why the immune system in people with SLE starts to malfunction. Also, some of the mutated genes are contained in the X chromosome. Chromosomes are large blocks of genetic information. Men only have one X chromosome (and one Y chromosome) whereas women have a pair of X chromosomes. Therefore, women are twice as likely to receive one of the mutated genes. This may partly explain why SLE is more common in women than men.

Environmental factors
A number of environmental factors may be responsible for triggering SLE in vulnerable individuals. There is limited evidence to suggest triggers may include:
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exposure to sunlight hormonal changes that occur during a womans lifetime, such as during puberty or pregnancy medications which can trigger drug-induced lupus. This generally goes away once you stop using the medication

Epstein-Barr virus (EBV) has also been suggested as a possible cause of SLE. However, EBV is a common viral infection that does not usually cause symptoms and in some people can cause glandular fever.

Diagnosing lupus
Systemic lupus erythematosus (SLE) can be a difficult condition to diagnose. This is because symptoms of SLE are sometimes similar to several other conditions, most of which are far more common than SLE. Diagnosis may also be difficult because symptoms can vary greatly from person to person. They may also change over time. For example, there may be periods where your symptoms are not very noticeable, or times when they

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flare up and become more severe.

Blood tests
If your GP suspects SLE after examining your symptoms, they will refer you for blood tests to help confirm the diagnosis. Some blood tests that may be carried out are listed below. Anti-nuclear antibody test An anti-nuclear antibody test checks whether there is a certain type of antibody cell in your blood, known as the anti-nuclear antibody. Approximately 95% of people with SLE have this antibody. However, it is possible to have the anti-nuclear antibody without having SLE, so the anti-nuclear antibody test is not a definitive way of testing for the condition. Your GP will need other tests to confirm the diagnosis. Anti-DNA antibody test An anti-DNA test also checks for a certain type of antibody in your blood. If you have the anti-DNA antibody, it is highly likely you will have SLE. However, the antibody can also be present in people who do not have the condition. The level of anti-DNA antibodies increases when SLE is more active, so during a flare-up of symptoms your reading from this test may be greater than normal. Complement level test Complement is a chemical in the blood that forms part of your immune system. Your GP may test the level of this chemical in your blood to check how active your SLE is. The level of complement in your blood decreases when SLE is more active.

Other tests
Once you have been diagnosed with SLE, you will normally need regular monitoring to see how the condition is affecting your body. If you have SLE it is possible you may develop other conditions, such as anaemia or kidney problems. Monitoring

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your condition will allow your doctor to check for these secondary conditions and, if necessary, treat them as soon as possible. You may need to have a scan, such as an X-ray, ultrasound scan or a computerised tomography (CT) scan to check whether SLE is affecting your internal organs.

Treating lupus
There is currently no cure for systemic lupus erythematosus (SLE). However, treatments are available that can help ease your symptoms and minimise the effect the condition has on your daily life.

Protecting yourself from the sun

Exposure to sunlight can sometimes make SLE symptoms such as skin rashes worse. Therefore, it is important to make sure you protect your skin when in the sun. This means wearing clothing that covers your skin, including a wide-brimmed hat and sunglasses. You will also need to apply sun screen with a high factor to prevent sunburn. However, some people with lupus are not sunsensitive and do not need to take precautions. If your SLE symptoms are mild, you may not need specific treatment. However, most people will require medication to help manage their symptoms. Some medicines you may need if you have SLE are described below.

Non-steroidal anti-inflammatory drugs (NSAIDs)

Non-steroidal anti-inflammatory drugs (NSAIDs) are a painkilling medication that reduces inflammation in the body. If you experience joint or muscle pain as a result of SLE, you may be prescribed a NSAID to help ease your symptoms.

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Commonly prescribed NSAIDs for SLE include:

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ibuprofen naproxen diclofenac piroxicam

You can buy some NSAIDs, such as ibuprofen, over the counter. These NSAIDs may be suitable if your joint or muscle pain is mild. For more severe pain you will need stronger medication prescribed by your GP. NSAIDs may not be suitable for people who have stomach, kidney or liver problems or have had these problems in the past. They may also be unsuitable for people with asthma. Children under the age of 16 should not take aspirin. Your GP will advise about which NSAID is right for you. If taken in high doses or over long periods of time, NSAIDs can damage your stomach lining, which may cause internal bleeding. If you need to take NSAIDs on a long-term basis, your GP will carefully monitor your condition to ensure the medication is not causing any stomach bleeding. However, if this occurs other options are available.

Hydroxychloroquine
Hydroxychloroquine is a medicine previously used to treat malaria but is also effective in treating some of the symptoms of SLE, such as skin rashes, joint and muscle pain and fatigue. You will usually have to take hydroxychloroquine for 612 weeks before you notice any benefit. Most expert doctors recommend people with SLE take hydroxychloroquine on a long-term basis as a way of controlling their symptoms, helping to prevent flare-ups and to prevent development of more serious problems from lupus. Side effects of hydroxychloroquine are rare but may include:
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indigestion diarrhoea headaches skin rashes

Hydroxychloroquine may cause more serious side effects in a small number of people. For example, approximately one in 2,000 people taking this medicine may experience damage to their vision. It is worthwhile

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having a baseline eye check at your local ophthalmic optician but, as side effects are so rare, routine monitoring in an eye clinic is no longer recommended. Contact your GP or specialist immediately if you experience vision problems while taking hydroxychloroquine. If your GP or specialist feels it is necessary, you may need regular eye examinations to help reduce your risk of developing complications.

Corticosteroids
Corticosteroids are a type of medicine that help reduce inflammation quickly. They are very effective but usually only prescribed if you have severe SLE. If you have severe symptoms of SLE, or if you are experiencing a flare-up, you may be given a large dose of corticosteroids to help bring your symptoms under control. As your symptoms ease, your dosage can gradually be reduced. When prescribing corticosteroids, the lowest effective dosage is always given. This is because high doses or long-term use of corticosteroids can cause side effects. These may include:
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thinning of your bones thinning of your skin weight gain high blood pressure (hypertension)

Corticosteroids are a safe and effective form of treatment provided they are taken correctly and under the supervision of your GP or specialist. They will tailor the steroid dose to your disease activity to minimise side effects while effectively controlling the condition.

Immunosuppressants
Immunosuppressants are a type of medicine that suppress your immune system. They can help improve your symptoms of SLE by limiting the damage your immune system causes when it attacks healthy parts of your body. Commonly prescribed immunosuppressant medicines include:
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azathioprine mycophenolate mofetil

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cyclophosphamide

Immunosuppressants are sometimes used in conjunction with corticosteroids (see above) because these medicines may ease your symptoms more effectively when used together. In addition, use of immunosuppressant medication may reduce the dose of corticosteroids needed. Immunosuppressant medication is usually only prescribed if you have severe SLE. This is because this type of medication is very powerful and can cause side effects such as:
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loss of appetite vomiting diarrhoea swollen gums bruising or bleeding more easily convulsions headache acne extra hair growth weight gain

The exact side effects will depend on which immunosuppressant is prescribed. Tell your GP if a side effect becomes particularly troublesome because your dose may need adjusting. Mycophenolate and cyclophosphamide can cause birth defects in women, so you should use a reliable form of contraception if you are taking these medications and are sexually active. If you are trying to become pregnant, an alternative medication such as azathioprine can be used. If possible, pregnancy should be planned in consultation with your specialist during a time when SLE is in remission (a period when there is a decrease in symptoms). Close monitoring by your specialist and your obstetrician will be essential during pregnancy. Suppressing your immune system increases your risk of developing infections. Immunosuppressants can also sometimes cause liver damage. For these reasons, you will need regular check-ups and blood tests when taking immunosuppressant medication. Report any symptoms of an infection to your GP immediately, because you may need prompt treatment to prevent serious complications.

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Symptoms of infection may sometimes be similar to a flare up of lupus and include:

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high temperature (fever) of 38C (100.4F) or above new onset of cough, phlegm or breathlessness burning on passing urine or blood in the urine (haematuria) diarrhoea

You should avoid contact with anyone known to have an infection even if it is an infection you were previously immune to, such as chickenpox or measles. This is because your previous immunity to these conditions will probably be suppressed (lowered).

Rituximab
Rituximab is a new type of medication used in people with SLE who don't respond to other treatments. Rituximab was originally designed to treat certain types of blood cancer, such as lymphoma. However, it has since proved effective in treating a number of autoimmune conditions, such as SLE and rheumatoid arthritis. Rituximab works by locking on to and killing the B-cells, which produce antibodies responsible for the symptoms of SLE. It is administered directly into your vein over the course of several hours, known as an infusion. Common side effects of rituximab include:
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flu-like symptoms, such as chills and a high temperature while the medication is being given dizziness vomiting

In rare cases, rituximab can cause infusion reactions. Most infusion reactions occur during or shortly after administration of the infusion treatment. You will therefore be closely monitored once your treatment begins. It is important to stress that rituximab is currently unlicensed for treating SLE in the UK. People with the condition should be aware there are uncertainties about how effective or safe rituximab is in treating SLE. However, although rituximab is unlicensed, there may be instances where your specialist may consider it an appropriate treatment for you.

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Expert Patients Programme

Many people with SLE have reported that they found joining the Expert Patient Programme (EPP) very helpful. The Expert Patients Programme (EPP) is an NHS-run self-management programme for people living with a chronic (long-term) condition. The aim is to support people who have a chronic condition by:
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increasing their confidence improving their quality of life helping them manage their condition more effectively

Complications of lupus
If your symptoms of systemic lupus erythematosus (SLE) are mild or well-controlled, you may find it barely affects your day-to-day life and you may not have complications. You may also find your symptoms significantly improve with age. Many people over 50 find their symptoms have eased. In some (but not all) women, SLE may improve after the menopause (when a womans monthly periods stop, usually at around the age of 50). However, for some people SLE can be a more serious condition which can cause life-threatening complications. Some of these complications are outlined below.

Cardiovascular disease
Cardiovascular disease (CVD) is a general term for any type of health condition that affects the heart and arteries. Examples of CVD include:
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high blood pressure (hypertension) atherosclerosis hardening and narrowing of the arteries coronary heart disease angina chest pain due to the heart not receiving enough blood heart attack strokes

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It is estimated that people with SLE are seven to eight times more likely to develop cardiovascular disease than the general population. This is because SLE can cause inflammation of your heart and arteries. It helps to take steps to lower your risk of developing CVD, including:
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quitting smoking (if you smoke) eating a healthy, balanced diet low in saturated fat, sugar and salt and containing plenty of fruit and vegetables (at least five portions a day) maintaining a healthy weight taking regular exercise at least 150 minutes a week (the exercise should be strenuous enough to leave you slightly out of breath) if you drink, make sure you stick to recommended guidelines for alcohol consumption (see below)

Maximum recommended daily limits of alcohol consumption are:

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three to four units of alcohol for men two to three units of alcohol for women

A unit of alcohol is equal to about half a pint of normal strength lager, a small glass of wine or a pub measure (25ml) of spirits. Read about healthy hearts for more information on how to prevent CVD. In some people, SLE can cause a number of less serious heart conditions such as:
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pericarditis inflammation of the lining of your heart myocarditis inflammation of the muscles of your heart

Lupus nephritis
Lupus nephritis is a potentially serious kidney disease caused by prolonged inflammation of your kidneys as a result of SLE. Lupus nephritis is a common complication of SLE, estimated to affect around half of people with the condition. Lupus nephritis tends to develop relatively early in the course of SLE, usually within five years of diagnosis. Symptoms of lupus nephritis include:

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swelling of your feet headaches dizziness blood in your urine a frequent need to urinate

In many cases, lupus nephritis does not cause any noticeable symptoms. However, this does not mean the condition is not dangerous as it can damage kidneys. Lupus nephritis can also cause high blood pressure (hypertension), which if not treated can put you at risk of developing a serious CVD in the long-term, such as a heart attack or stroke. It is likely you will be referred for regular blood tests so the condition of your kidneys can be carefully monitored. If you develop lupus nephritis, it can usually be successfully controlled using immunosuppressants such as azathioprine, mycophenolate mofetil or cyclophosphamide.

Other autoimmune conditions

Approximately one in three people with SLE also have another autoimmune condition, such as thyroid disease, Sjogrens syndrome or Hughes syndrome (antiphospholipid syndrome). Sjogren's syndrome Sjogrens syndrome is a condition that damages your salivary and tear glands, causing a dry mouth and eyes. It affects one in eight people with SLE. Hughes syndrome (antiphospholipid syndrome) Hughes syndrome (antiphospholipid syndrome) can occur as a secondary condition to SLE or on its own. Hughes syndrome causes an increased risk of clots developing in the arteries (leading to strokes and heart attacks) and the veins (leading to deep vein thrombosis). Women with SLE and Hughes syndrome also have an increased risk of developing complications during pregnancy (see below). Diagnosis of Hughes syndrome (antiphospholipid syndrome) in cases of SLE requires the presence of either vascular and/or pregnancy-related complications together with antiphospholipid antibodies in the blood.

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Lupus in pregnancy
SLE does not usually affect fertility. Pregnancy problems are more common in people with lupus, especially those who have Hughes syndrome (see above). Complications occurring during pregnancy include:
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pre-eclampsia miscarriage stillbirth premature delivery

Treatments such as aspirin and heparin injections are available, which increase the chance of a successful pregnancy in patients with Hughes syndrome. There is also a risk that women with lupus will experience a flare-up of their symptoms during pregnancy. Symptoms of SLE that can develop during pregnancy include:
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hair loss swelling of the feet and hands facial redness anaemia muscle, bone and joint pain

Some symptoms may be difficult to distinguish from those related to pregnancy itself, such as anaemia, facial redness and swelling. The risk of flare-ups can usually be reduced by taking anti-malarial medications and appropriate immunosuppressants, such as azathioprine. If necessary, corticosteroids can also be given. To reduce the risks of complications developing during pregnancy you may be advised to delay your pregnancy until you have gone for six months without experiencing a flare-up of your symptoms and your kidney function is normal or nearly normal. While no medication is guaranteed safe to use during pregnancy, medications known to be safest are:
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hydroxychloroquine an anti-malarial medication azathioprine an immunosuppressant prednisolone a corticosteroid

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Read more information about these on our medicines information page for lupus.

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