Lupus

Lupus is a complicated condition, and doctors still don't fully understand it. It can affect many parts of the body. The symptoms vary a great deal, from mild to life-threatening.

Some types of lupus affect only the skin, such as discoid lupus erythematosus and subacute cutaneous lupus erythematosus. Some medicines can also cause lupus-like side effects.

But when people say "lupus", they usually mean a more severe form called systemic lupus erythematosus (SLE). It can affect many parts of the body, including the skin, joints and internal organs.

Symptoms can be mild or severe. Many people have long periods with few or no symptoms, and then a sudden flare-up, when their symptoms are particularly severe.

Even mild cases can be distressing and can have a considerable impact on a person's quality of life.

The rest of this article will focus on SLE.

Lupus (SLE) can cause many different symptoms, depending on which parts of the body are affected. The most common ones are:

• fatigue (extreme tiredness)
• rashes, particularly on the face, wrists and hands
• joint pain and swelling

SLE can be hard to diagnose, because its symptoms look like those of a number of other conditions, and many of those are more common.

If you have symptoms that don't go away or keep troubling you, and you think SLE could be the cause, see your doctor so they can try to find out what is behind them.

SLE (systemic lupus erythematosus) is an autoimmune condition, which means the problem lies in the immune system. In people with lupus, the immune system starts to attack and inflame healthy cells, tissue and organs. Why this happens isn't yet understood.

Other autoimmune conditions, such as rheumatoid arthritis, are more common, and they share something with SLE. It's thought that a combination of genetic and environmental factors may trigger SLE in certain people.

SLE (systemic lupus erythematosus, the most common form of lupus) is an uncommon condition, and around 90% of cases occur in women. It's most common in women of childbearing age, between the ages of 15 and 50, but people of other ages can get it too.

SLE tends to be less common in people of white European origin. It's more common in those of African, Caribbean or Asian origin.

There is no cure for SLE (systemic lupus erythematosus) at the moment. But several medicines can ease many of the symptoms and lower the chance of damage to the organs.

These medicines include:

• hydroxychloroquine, a medicine that has long been used to treat malaria but can also help with some symptoms of SLE
• corticosteroids, which are anti-inflammatory medicines
• immunosuppressants, a group of medicines that suppress your immune system

With good support from friends, family and healthcare professionals, many people with SLE are able to manage their condition well.

A few decades ago, SLE (systemic lupus erythematosus, the most common form of lupus) was seen as a terminal condition, because many people died of a related complication within a few years of diagnosis.

Things are much better now, thanks to earlier diagnosis and advances in treatment. The vast majority of people diagnosed with SLE will have a normal or near-normal life expectancy.

Even so, some people with SLE are still at risk of life-threatening complications, because the condition can damage internal organs and tissues. A heart attack or stroke is an example. SLE, and some of the treatments for it, can also raise your risk of developing potentially serious infections.

Read more about the complications of lupus.

The symptoms of systemic lupus erythematosus (SLE) differ a great deal from one person to the next. Some people have only a few mild symptoms, while others are affected much more severely.

Even if your symptoms are usually mild, SLE can "flare up". During a flare, your symptoms become worse or new ones appear.

Lupus, also called SLE, has 3 main symptoms: fatigue, joint pain and rashes.

Tiredness is one of the most common symptoms of lupus (SLE). You may feel very tired even when you've had plenty of sleep. Everyday tasks, like housework or office work, can leave you exhausted.

Many people with SLE say the fatigue is the most distressing and disruptive part of the illness, because it affects their work and their social life.

If you have SLE (systemic lupus erythematosus), joint pain in your hands and feet is likely. The pain can shift from one set of joints to another quite quickly, and it's usually worse in the morning.

Some other joint conditions leave the joints permanently damaged or deformed. SLE is unlikely to do this.

Many people with SLE (systemic lupus erythematosus) get rashes on their skin, most often on the face, wrists and hands. A rash across the cheeks and the bridge of the nose is especially common. It's known as a "butterfly rash" or "malar rash".

Rashes caused by SLE may clear up after a few days or weeks, but they can last longer and sometimes they become permanent.

These rashes can also be itchy or painful, and sunlight may make them worse. This sensitivity to sunlight is known as "photosensitivity".

SLE (systemic lupus erythematosus, or lupus) can cause many other symptoms too. You're unlikely to have all of the ones listed below, though, and many people with the condition only get the main symptoms.

Other features of SLE may include:

• a fever (high temperature)
• swollen lymph glands (small glands found throughout your body, including in your neck, armpits and groin)
• recurring mouth ulcers
• hair loss (alopecia)
• high blood pressure (hypertension)
• headaches and migraines
• stomach (abdominal) pain
• chest pain
• depression
• dry eyes
• memory loss
• seizures (fits)
• problems thinking clearly and difficulty telling the difference between reality and imagination (psychosis)
• 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)

See a doctor if you have symptoms that keep coming back or that trouble you, and you think they could be caused by lupus (SLE). Your symptoms are most likely caused by a more common condition. Even so, you need to see a doctor to find out for sure.

Read more about diagnosing lupus.

Systemic lupus erythematosus (SLE) is an autoimmune condition, which means problems with the immune system cause it.

The immune system is the body's natural defence against illness and infection. When it detects an infectious agent, such as bacteria or a virus, it sends white blood cells and antibodies to attack it. In SLE, the antibodies the immune system releases can attack healthy tissue, cells and organs instead.

Nobody knows exactly why this happens. Most experts think SLE has more than one cause. It has been suggested that a number of genetic factors may make some people more likely to develop the condition, and that one or more environmental factors are then needed to trigger lupus in those who are susceptible.

Possible genetic and environmental factors are discussed in more detail below.

If you have a brother or sister with SLE, you are much more likely to develop the condition than most people in the population.

Researchers have found a number of different genetic mutations that seem to make people more susceptible to SLE. A mutation happens when the normal instructions carried in certain genes become "scrambled", and the body's processes then don't work as they should.

Most of the faulty genes are linked to the way the immune system is regulated. That may explain why the immune system in people with lupus starts to malfunction.

Several things in a person's surroundings or life may set off SLE (the most common form of lupus) in people who are already vulnerable to it, though the evidence for many of these is limited.

The factors that have been suggested include:

• exposure to sunlight (ultraviolet light)
• hormonal changes that happen during a woman's lifetime, such as during puberty or pregnancy
• certain infections, such as Epstein-Barr virus (EBV), a common viral infection that doesn't usually cause any symptoms
• smoking

Systemic lupus erythematosus (SLE) can be hard to diagnose, because its symptoms look like those of several other conditions that are far more common.

The symptoms also differ a great deal from one person to the next, and they can change over time. You may have periods when your symptoms are barely noticeable, and other times when they flare up and become more severe.

To be confident of an SLE diagnosis, a doctor needs to see that you have several symptoms of lupus. A number of blood tests may also be needed.

Several blood tests may be done if your doctor suspects lupus. Some of them are described below.

A blood test called the erythrocyte sedimentation rate (ESR) test can show whether there is any inflammation in your body. This helps when a doctor is looking for SLE (lupus), because the condition can make many parts of the body inflamed (swollen), including the joints and internal organs.

The test measures how long it takes for red blood cells to fall to the bottom of a test tube. The quicker they fall, the more likely it is that the level of inflammation is high.

An anti-nuclear antibody test checks your blood for a certain type of antibody cell, called the anti-nuclear antibody. About 95% of people with SLE (lupus) have this antibody.

But you can have the anti-nuclear antibody and still not have SLE, so the test can't settle the question on its own. You will need other blood tests as well to confirm the diagnosis.

An anti-DNA test looks for a particular antibody in your blood, called the anti-DNA antibody. If you have it, it's highly likely that you have SLE (systemic lupus erythematosus). But the antibody is found in only around 70% of people with the condition, so not having it doesn't rule SLE out.

The level of anti-DNA antibodies goes up when SLE is more active. So during a flare-up of symptoms, your reading from this test may be higher than normal.

Complement is a chemical in your blood, and it's part of your immune system. Doctors may test the level of complement to check how active your SLE (systemic lupus erythematosus, the most common form of lupus) is. When SLE is more active, the level of complement in your blood goes down.

Once your doctor has diagnosed SLE, you will normally need regular monitoring to see how the condition is affecting your body.

People with SLE can go on to develop other conditions, such as kidney problems. Regular check-ups let your doctor look for these complications and, if necessary, treat them as soon as possible.

You may also need scans to check whether SLE is affecting your internal organs. These can include an X-ray, an ultrasound scan, a magnetic resonance imaging (MRI) scan or a computerised tomography (CT) scan.

Read more about the complications of lupus.

There's no cure for systemic lupus erythematosus (SLE) at the moment. But there are treatments that can ease the symptoms and make life with the condition easier.

For most people, treatment means a mix of self-care and medicine.

Sunlight can sometimes make lupus symptoms, such as rashes, worse, so you need to look after your skin when you're out in the sun.

That means wearing clothes that cover your skin, a wide-brimmed hat and sunglasses. You'll also need a sunscreen with a high SPF to prevent sunburn. Not everyone with lupus is sensitive to the sun, though, and those people don't need to take extra precautions.

Most of our vitamin D comes from direct sunlight on the skin. If you have to stay out of the sun, you may not get enough of it. So you may need to make an extra effort to eat good sources of vitamin D, to avoid problems such as osteoporosis (weakened bones), and your doctor may advise you to take vitamin D supplements.

Non-steroidal anti-inflammatory drugs (NSAIDs) are a common type of painkilling medicine that reduces inflammation in the body. If lupus (SLE) gives you joint or muscle pain, your doctor may prescribe an NSAID to help ease it.

The NSAIDs most often prescribed for SLE are ibuprofen, naproxen and diclofenac.

You can buy some NSAIDs, such as ibuprofen, over the counter. These may be enough if your joint or muscle pain is mild. For more severe pain, you will need a stronger medicine prescribed by your doctor.

NSAIDs may not be suitable if you have stomach, kidney or liver problems, or have had them in the past. They may also be unsuitable for people with asthma. Your doctor will advise you on which NSAID is right for you.

NSAIDs can damage your stomach lining if you take them in high doses or over a long period, and that may cause internal bleeding. If you need to take NSAIDs for a long time, your doctor will keep a close watch for any problems. You may also be prescribed another medicine, called a proton pump inhibitor (PPI), to protect your stomach.

Hydroxychloroquine is a medicine that was first used to treat malaria, but it also works well against some of the symptoms of SLE (systemic lupus erythematosus), such as rashes, joint and muscle pain, and fatigue.

You will usually have to take hydroxychloroquine for 6 to 12 weeks before you notice any benefit.

Most expert doctors recommend that people with SLE take hydroxychloroquine in the long term. It helps control symptoms, helps prevent flare-ups, and helps prevent more serious problems from lupus developing.

Side effects from hydroxychloroquine are uncommon, but they can include indigestion, diarrhoea, headaches and rashes.

In a small number of people, the medicine can cause more serious problems. In rare cases, for example, it can damage the eyes. If you notice any problem with your vision while you're taking hydroxychloroquine, contact your doctor or specialist immediately.

If your doctor or specialist thinks it's necessary, you may need to have your eyes examined regularly.

Corticosteroids are medicines that bring down inflammation quickly. In lupus (SLE), they can work very well on the symptoms, but doctors usually prescribe them only when the condition is severe.

If your SLE symptoms are severe, or you're having a flare-up, you may be given a large dose of corticosteroids to get the symptoms under control. Once the symptoms start to ease, your doctor can lower the dose bit by bit.

When doctors prescribe corticosteroids for lupus, they always give the lowest dose that works. High doses, or taking the medicine for a long time, can cause side effects. These may include:

• thinning of your bones
• thinning of your skin
• weight gain
• high blood pressure (hypertension)

Corticosteroids are a safe and effective treatment, as long as you take them correctly and under the supervision of your doctor or specialist. They will adjust the steroid dose to match how active your disease is, so that side effects stay as low as possible while the condition is still kept under control.

Immunosuppressants are medicines that hold back your immune system. In lupus (SLE), your immune system attacks healthy parts of your body, and these medicines can help improve your symptoms by limiting the damage it causes.

Doctors commonly prescribe azathioprine, methotrexate, mycophenolate mofetil and cyclophosphamide.

Sometimes an immunosuppressant is given together with corticosteroids (see above), because the two may ease your symptoms more effectively when used together. Or the immunosuppressant may make it possible to lower your corticosteroid dose.

Doctors usually prescribe immunosuppressant medicine only if you have severe SLE (systemic lupus erythematosus). These medicines are powerful, so they can cause side effects such as:

• loss of appetite
• vomiting
• diarrhoea
• swollen gums
• bruising or bleeding more easily
• headache
• acne
• extra hair growth
• weight gain
• liver damage
• an increased risk of infection (see below)

Methotrexate, mycophenolate mofetil and cyclophosphamide can also cause birth defects if you take them during pregnancy. So if you are sexually active and taking any of these medicines, use a reliable form of contraception. If you are trying to become pregnant, a different medicine such as azathioprine can be used instead.

If a side effect becomes particularly troublesome, tell your doctor. It may mean your dose needs to be adjusted.

Taking immunosuppressant medicine can raise your risk of getting an infection. For people with SLE (lupus) this is a particularly serious concern. The condition can damage organs, and that makes an infection more likely to threaten your life.

So if you notice any sign of a possible infection, tell your doctor straight away.

Signs of infection can sometimes look like a flare up of SLE. They include:

• a fever (high temperature)
• a cough or breathlessness
• burning when passing urine or passing blood in your urine
• diarrhoea

Try to stay away from anyone known to have an infection, even one you were immune to before, such as chickenpox or measles. Your earlier immunity to these illnesses will probably be suppressed (lowered).

Rituximab is a fairly new medicine for people with severe SLE (systemic lupus erythematosus) that hasn't responded to other treatments.

It was first made to treat certain cancers, such as lymphoma. Since then it has proved effective for a number of autoimmune conditions, including SLE and rheumatoid arthritis.

Rituximab locks on to cells called "B-cells" and kills them. These cells produce the antibodies that are responsible for the symptoms of SLE. The medicine is given straight into a vein over several hours, which is called an infusion.

Rituximab is not currently licensed for treating SLE, but your specialist may still think it is the right treatment for you. If your doctor suggests it, they should tell you that there are some uncertainties at present about how effective or safe it is for SLE.

Rituximab can cause some common side effects, including:

• flu-like symptoms, such as chills and a high temperature
• dizziness
• vomiting

In rare cases, rituximab causes a more serious allergy-like reaction. Most reactions happen during the treatment or soon after it, so the medical team will watch you closely once your treatment begins.

Belimumab is a newer medicine for people with active SLE who don't respond to other treatments.

It works by binding to growth factors that B-cells need to survive. You get it directly into a vein over several hours, which is called an infusion. The first 3 doses are given 14 days apart, and after that the medicine is usually given once a month.

Belimumab is licensed for treating SLE in the UK, and there may be times when your specialist considers it the right treatment for you. If your doctor suggests belimumab, they should tell you that there are currently some uncertainties about how effective or safe it is for SLE.

Belimumab, a medicine used for lupus, can cause some common side effects:

• flu-like symptoms, such as chills and a high temperature
• nausea and diarrhoea
• headache
• difficulty sleeping (insomnia)
• a cough, sore throat and blocked or runny nose
• joint pain
• increased risk of infections
• changes in blood pressure

Rarely, belimumab causes a more serious allergy-like reaction. Most reactions happen during the treatment or soon after it's given, so you will be watched closely once your treatment begins.

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 any complications at all.

For some people, though, SLE is more serious and can cause life-threatening complications. Some of these are outlined below.

Around 1 in every 3 people with SLE will develop a potentially serious kidney disease called lupus nephritis, which is caused by long-lasting inflammation of the kidneys. It tends to appear fairly early in the course of SLE, usually within five years of diagnosis.

Symptoms of lupus nephritis can include:

• swelling of your feet (oedema)
• headaches
• dizziness
• blood in your urine
• a frequent need to urinate

Lupus nephritis can also cause high blood pressure (hypertension). If it's left untreated, you could be at risk of life-threatening problems such as a heart attack or stroke.

In many cases, though, lupus nephritis causes no symptoms you would notice. That doesn't mean it isn't dangerous, because the kidneys could still be getting damaged.

If you have SLE, you will probably need regular blood tests so your doctor can keep a close watch on how your kidneys are doing. If you do develop lupus nephritis, it can usually be controlled successfully with immunosuppressants such as mycophenolate mofetil or cyclophosphamide.

In a small number of cases, the kidney damage becomes severe enough to need dialysis (where a machine does many of the kidneys' jobs) or a kidney transplant.

Cardiovascular disease (CVD) is a general term for any health condition that affects the heart and arteries. It is often linked with blood clots and atherosclerosis, which means hardening and narrowing of the arteries.

Examples of CVD include coronary heart disease, angina (chest pain caused when the heart doesn't get enough blood), heart attack and stroke.

If you have SLE, you are more likely to develop CVD than the general population, because SLE can cause your heart and arteries to become inflamed and damaged.

You can lower your risk of CVD by making healthy lifestyle changes, such as:

• stopping smoking if you smoke
• eating a healthy, balanced diet low in saturated fat, sugar and salt, and containing at least five portions of fruit and vegetables a day
• maintaining a healthy weight
• exercising regularly, with at least 150 minutes (two-and-a-half hours) a week of exercise strenuous enough to leave you slightly out of breath being recommended
• cutting down on your alcohol consumption

SLE (systemic lupus erythematosus, the most common form of lupus) does not usually affect fertility. It can, though, raise your risk of pregnancy complications, such as:

• pre-eclampsia (a condition that causes high blood pressure during pregnancy)
• premature delivery
• miscarriage
• stillbirth

Some children of women with SLE are also born with heart block (where the electrical pulses that control the beating of the heart are disrupted) and rashes. This is known as "neonatal lupus syndrome".

If you have SLE and are thinking of having a baby, it's best to plan this carefully with your doctors if you can. The risk of complications is higher if you become pregnant while your symptoms are particularly severe, so you will usually be advised to try to avoid getting pregnant until your symptoms are better controlled.

If you do become pregnant, your specialist and an obstetrician will need to monitor you closely, so they can check for any problems.