What is lupus?
Lupus is an autoimmune disease, which means that the immune system, your body's defence system, produces antibodies that attack the body's own tissues, causing inflammation. There are two main types of lupus:
- discoid lupus
- systemic lupus erythematosus (SLE).
These pages focus on SLE, which is often just known as lupus.
Who gets lupus?
- about nine times as common in women as in men
- more common in younger people – only about 1 in 15 cases begin after the age of 50, when it tends to be less severe
- more common in women of Chinese origin
- most common in women of African or Caribbean origin. It tends to be more severe in people of Afro-Caribbean origin.
Rarely, lupus can affect children, but it's unusual before the age of five.
The most common symptoms of lupus are:
- joint pains
- skin rashes
- extreme tiredness (fatigue).
Some people with lupus will only have these symptoms, though they can still have a big impact on daily life.
Other symptoms of lupus which can be quite common are:
- weight loss
- swelling of the lymph glands.
Lupus can affect many different parts of the body, and when internal organs such as the heart, lungs, brain or kidneys are involved it can be much more serious. But most people will only have one or a few of the possible symptoms, and many people will find that the symptoms come and go.
Skin and mouth
It's common for a rash to develop over parts of the body that are exposed to the sun, including the face, wrists and hands. A butterfly-shaped rash over the cheeks and the bridge of the nose is especially common.
Some people with lupus notice that their fingers change colour in cold weather, going first very pale, then blue and finally red. This is called Raynaud's phenomenon and is caused by narrowing (constriction) of the blood vessels, which reduces the blood supply to the fingers or toes.
You may develop groups of mouth ulcers, which may come back repeatedly.
Some hair loss is common and can be severe in some people with lupus, but once a flare-up is brought under control the hair will usually grow back.
Joint pain is common in lupus, especially in the small joints of the hands and feet. The pain tends to move from joint to joint and is often described as 'flitting'.
Joint pain and swelling can be the main symptoms for some people, though lupus doesn't usually cause joints to become permanently damaged or deformed.
About 1 in 20 people with lupus develop more severe joint problems. Fewer than 1 in 20 have joint hypermobility or a form of arthritis called Jaccoud’s arthropathy, which can change the shape of the joints.
Around one in three people with lupus have significant inflammation of the kidneys, and kidney damage can sometimes occur. Kidney inflammation can be treated successfully in most patients if it's identified early with regular urine, blood pressure and blood testing by your doctor.
You must take any medication as prescribed by your doctor to make sure that your kidneys aren't permanently damaged.
Blood and blood vessels
Lupus can cause high blood pressure, particularly if the kidneys are involved. Steroid tablets, which are often used to treat lupus, can raise blood pressure particularly when used in high doses. Lupus can contribute to the development of high cholesterol, which should be checked yearly with a blood test and treated if necessary.
Lupus may also affect the bone marrow, causing amaemia and a reduction in the number of platelets (cells that help the blood to clot) and/or white blood cells. Blood-related problems like anaemia tend to be more common in children with lupus.
Some people with lupus are more at risk of developing blood clots in veins or arteries. This problem is usually caused by antiphospholipid antibodies. Some of these autoantibodies can also affect pregnancy, causing an increased risk of miscarriage (antiphospholipid syndrome).
Brain and nervous system
As many as one in three people with lupus may have migraines and may experience anxiety or depression. Some people have diziness, memory loss or confusion. Rarely, lupus can cause fits (similar to epilepsy) or feelings of paranoia (similar to schizophrenia) - though these complications only affect a small number of people with lupus.
Heart and lungs
Occasionally, lupus directly affects the heart and lungs. More often, it causes inflammation in the lining tissues around the heart (pericarditis) and lungs (pleurisy), both of which cause breathlessness and sharp pains in the chest. Rarely, large amounts of fluid develop in these lining layers, causing severe breathlessness.
More recently, we've found that lupus may also cause narrowing of the blood vessels. This can lead to increased risk of angina, heart attacks and strokes, so close monitoring and early treatment of factors such as high cholesterol and high blood pressure are vital.
People with lupus can suffer swelling of the lymph glands, which may cause discomfort.
Less frequently, lupus can affect the lining tissue of the gut (serositis), the gut, pancreas, liver or spleen, causing pain in the abdomen. Very rarely, lupus can affect the eyes, causing a painful red eye or changes in the eyesight.
About one third of people with lupus develop an additional autoimmune disease. Examples include autoimmune thyroid disease, in particular the type which makes the thyroid gland underactive.
Severe dryness of the eyes and mouth (Sjögren’s syndrome) occurs in about 10% of people with lupus, and rheumatoid arthritis or inflammation of your muscles (myositis) can develop, but these are much less common.
Your immune system makes proteins called antibodies that fight infection. In lupus, the body also makes autoantibodies that attack the body's own tissues. We're not sure why this happens – but it's probably due to a combination of environmental, hormonal and genetic factors.
Lupus isn't directly passed on from a parent to their children, but if you have a close relative with lupus you're at increased risk of developing it. Similarly, if you have lupus there's about a 1 in 100 chance of your child developing it in later life.
Lupus isn't contagious, so you can't catch it from anyone else.
How will lupus affect me?
Modern treatments have improved the outlook for people with lupus. However, it remains a variable and unpredictable condition and may even be life-threatening for people whose vital organs are affected.
It's hard to predict exactly how lupus will affect you. Most people with lupus don't have the more serious complications, but your doctor and rheumatology nurse specialist will be on the look-out for these so that early treatment can be given if necessary.
Careful monitoring of the condition is needed so that potentially serious complications can be recognised and treated promptly. For example, some people with lupus will have a greater than usual risk of having a heart attack or stroke. Your doctor will take this into account, along with any other risk factors, such as smoking and raised cholesterol or blood pressure. They'll suggest treatments and lifestyle changes.
In young women, the risk of a heart attack or stroke would normally be very low, so although it's increased by having lupus, the overall risk is still low. Even so, it's a good idea to think about and discuss this with your doctor.
A diagnosis of lupus is made based on symptoms, a physical examination and blood tests. Tests can help to rule out other conditions.
A number of different blood tests may be used:
- Anti-nuclear antibody (ANA) test. About 95% of people with lupus are ANA positive, but the test can sometimes be positive in people who don't have lupus, so it can’t confirm the diagnosis.
- Anti-double-stranded DNA (anti-dsDNA) antibody test. About 70% of people with lupus have these antibodies. A positive test means that lupus is highly likely as the test is hardly ever positive in people who don't have lupus. The anti-dsDNA level usually goes up when lupus is more active, so repeat tests may be helpful as a means of monitoring your condition and deciding on treatment.
- Anti-Ro antibody test. If you test positive for this autoantibody you may be more likely to get skin rashes and suffer from dry eyes or a dry mouth (Sjögren's syndrome). This autoantibody can pass across the placenta during pregnancy. If you carry the anti-Ro autoantibody and decide to have a baby, your pregnancy will be more closely monitored.
- Antiphospholipid antibody test. A positive test for these autoantibodies may mean an increased risk of miscarriage and developing blood clots.
- Complement level test. Complement refers to a set of proteins in the blood that protect us from infections. Complement levels go down when lupus is more active.
- Erythrocyte sedimentation rate (ESR) test. This test assesses inflammation by measuring how quickly the blood cells settle at the bottom of a test tube. The ESR is often raised in lupus.
- Kidney and liver function tests. These include blood and urine tests, which are carried out regularly so any problems caused by lupus itself or by drug treatment can be recognised and dealt with quickly. A simple urine test can show if there's protein or blood in the urine. This test can help doctors recognise a problem in the kidneys at a very early stage. Further tests, such as kidney filtration tests, may be carried out if necessary. Those with kidney test abnormalities may be asked to have a biopsy which is the most accurate way of finding out how inflamed and damaged the kidneys are.
- Blood cell counts. Haemoglobin, white and red blood cells and platelets are all made in the bone marrow, so blood cell counts can help to show whether the bone marrow is affected, either by the disease or the drugs you're using to treat it.
These tests can also be helpful in monitoring the condition after diagnosis – for example, a combination of higher levels of anti-dsDNA and falling complement levels (often accompanied by a high ESR test) is helpful in predicting a flare-up of lupus. If the C-reactive protein (CRP), another measure of inflammation, is raised, your doctor would also consider whether you have an infection.
A variety of tests are available to check how your heart, lungs, liver and spleen are working. Depending on which organs your doctor thinks may be involved, you may have x-rays, an ultrasound scan, computerised tomography (CT) scan or a magnetic resonance imaging (MRI) scan.
A urine test can show if there's protein or blood in the urine. This can help doctors to recognise a problem in your kidneys at a very early stage. You may need further tests, such as kidney filtration tests.
If you have symptoms such as fever, weight loss and persistent swelling of the lymph glands, your doctor may take a biopsy of lymph gland tissue to rule out cancer, which can also cause these symptoms.
There's no cure for lupus at present, but the condition is most often very treatable and usually responds well to a number of different types of drugs – especially when treatment is started in the early stages of the disease.
The drugs used to treat lupus will depend on the severity of your disease and which parts of your body are affected. Your treatment will probably be changed or adjusted as your symptoms flare-up up or improve.
Most of the drugs described below were originally developed for other diseases but were later found to be useful in lupus.
Two newer drugs are now sometimes used for treatment of severe lupus – rituximab and belimumab. These are biological therapies which act against B-cells – the blood cells which produce antibodies. Research is continuing to find out which patients respond best to these drugs.
Non-steroidal anti-inflammatory drugs
Examples: naproxen and ibuprofen
- reduce inflammation
- help with symptoms in your joints
- often used for short periods.
Read more about non-steroidal anti-inflammatory drugs (NSAIDs).
- useful for skin rashes.
- reduce inflammation
- used alone or with steroid creams for skin rashes
- also useful as a treatment for fatigue and joint pain
- can help to lower cholesterol and control kidney disease.
- used for short periods for complications such as pleurisy or pericarditis
- may also be used as a longer-term treatment for other problems, such as kidney inflammation or severe blood problems.
When taking steroid tablets you must carry a steroid alert card, which records your dose and how long you've been taking them. If you become ill, or are involved in an accident in which you're injured or become unconscious, it's important for the steroid to be continued. The dose might also need to be increased because the treatment may prevent your body from being able to produce enough natural steroids in response to stress, as normally happens in this situation. Your doctor, rheumatology nurse specialist or pharmacist can give you a steroid card.
Read more about steroid tablets.
Disease-modifying anti-rheumatic drugs (DMARDs)
- used to dampen down the overactive immune system
- may need to be taken for long periods, though the dose may be reduced if the disease becomes less active
- will often control high blood pressure and have the added benefit of helping to prevent kidney problems
- may be used along with steroid tablets to allow steroid dose to be reduced.
Examples: rituximab and belimumab
- remove or reduce the activity of B-cells (a type of white blood cell that produces harmful autoantibodies)
- used for patients with lupus when conventional DMARDs aren't effective, though research into their effect is continuing.
If you're being prescribed a biological therapy, such as rituximab, it's recommended that you carry a biological therapy alert card, which you can obtain from your doctor or rheumatology nurse specialist. Then if you become unwell, anyone treating you will know that you’re on a biological therapy and that you're therefore at risk of its side-effects, including injections.
- can be injected into a muscle or vein as immediate treatment to help control a flare-up
- can also be injected into the scalp if hair loss is a problem (though often the hair grows back by itself when the disease is brought under control).
Read more about steroid injections.
- may be used to control high blood pressure.
Treatments for Raynaud's phenomenon
Examples: nifedipine tablets and iloprost injections
- may be used to widen your blood vessels to improve circulation.
What about side-effects?
All drugs have some potential side-effects, and you and your doctor will need to balance the risk of side-effects with the need to control your symptoms. Sometimes this may mean taking additional medications to protect against the side-effects of others.
NSAIDs can sometimes have side-effects, but your doctor will take precautions to reduce the risk of these – for example, by prescribing the lowest effective dose for the shortest possible period of time.
NSAIDs can cause digestive problems, including:
- stomach upsets
- damage to the lining of the stomach.
In most cases, therefore, an additional drug called a proton pump inhibitor (PPI) will be prescribed, which will help to protect the stomach.
There is an increased risk of heart attack or stroke with taking NSAIDs. Your doctor will be cautious about prescribing NSAIDs, especially for long periods, if there are other factors that may increase your overall risk, for example:
- circulation problems
- high blood pressure
- high cholesterol
If you're taking steroid tablets at high doses or for long periods of time, you may be given bisphosphonates, calcium tablets and vitamin D tablets to guard against osteoporosis. You may be given DMARDs instead of high doses of steroids. These should be avoided, however, if you have lupus in your kidneys as DMARDs can cause further kidney problems.
If you have lupus, and especially if you're on a DMARD or biological therapy, you'll be more prone to infection. Take care to avoid close contact with people with active infectious diseases like chickenpox or people who have recently been in hospital.
You shouldn't have live vaccines if you're on more than 10 mg of prednisolone daily or if you're on certain DMARDs or biological therapies. These vaccines include:
- yellow fever
- live typhoid
- live oral poliomyelitis (polio).
You should think about this when you're planning a holiday. Check with your doctor if you think you might need these vaccinations.
Pneumovax (which gives protection against the most common cause of pneumonia) and yearly flu vaccines are well tolerated and recommended.
If there's a significant build-up of fluid in the lining tissues of your heart or lungs, this may need to be drained using a needle and syringe.
Rarely, some people with lupus develop kidney failure, which may require dialysis or a kidney transplant. However, severe kidney damage can usually be prevented by early diagnosis and treatment of lupus. It's important therefore to take any medications as prescribed by your doctor.
Managing your symptoms
Although drugs are important in controlling lupus, there's a lot you can do to help manage your symptoms. The following lifestyle factors will help you reduce your risk of developing the more serious complications of lupus:
- following a healthy diet
- the right balance of exercise and rest
- not smoking.
Managing a flare-up
Lupus is a condition that naturally improves and worsens at different times. Learning how to manage a flare-up of your symptoms lets you be more in control of your condition. The reasons for a flare-up can vary from person to person, but the following can play a part:
- exposure to sunlight
- too little rest
Try to identify the things that lead to a flare-up and find ways of managing or avoiding them.
Living with lupus
Too much ultraviolet light from sunlight can cause a red rash across the cheeks and the bridge of the nose, often known as the butterfly rash. It can also sometimes cause problems with internal organs to flare up. Bear this in mind when you choose a holiday destination and talk to your rheumatology nurse specialist or dermatologist if you're in doubt. When you're outside, try the following tips:
- Keep out of the midday sun and wear a hat.
- Cover your skin or use a sun-blocking cream, SPF 50 or greater. This is available on prescription for people with lupus. You can also buy high-factor sun creams that include a tint and can be used as foundation make-up.
- Be careful when sitting under sun umbrellas on a paved area because the sunlight will be reflected onto your face.
Pregnancy and lupus
Most women with lupus should be able to have a baby if they wish to, but it's best to discuss your plans with your doctor before trying to get pregnant so that your treatments can be altered if need be. Try to plan your pregnancy when your lupus is inactive and you're taking as little medication as possible.
A small number of women with very severe lupus may be advised against having a baby as pregnancy can put a great strain on the heart, lungs and kidneys. There's an increased risk of pregnancy complications in women whose lupus affects a number of different organs and who find it difficult to control their symptoms without certain medications.
If you're thinking of having a baby, always discuss your plans with your doctor or specialist nurse before you start trying. You may need to see an obstetrician with a special interest in lupus for further advice.
Doctors are naturally cautious about which drugs are used during pregnancy. Steroids are usually well tolerated and many people have used prednisolone, hydroxychloroquine and azathioprine throughout pregnancy without ill effects.
If you have high levels of antiphospholipid antibodies, there's an increased risk of miscarriage. However, treatment with aspirin and/or heparin reduces this risk, and there are now many more successful pregnancies in women who have these antibodies.
There's conflicting evidence about whether pregnancy is likely to cause a flare-up of lupus. If your condition is well controlled at the time the baby is conceived, and you don't have kidney disease, then you're unlikely to have any problems.
If you do have kidney disease, then you may well have increased protein in your urine during the later stages of pregnancy. It is important, but sometimes difficult, to distinguish between protein in the urine due to kidney disease which is associated with lupus, or to a more common complication of pregnancy, known as pre-eclampsia. The doctors looking after your pregnancy will check for this and consult your rheumatologist if they need to.
In pregnant women who have anti-Ro antibodies there's a small risk (about 1 in 50) that their babies will have neonatal (newborn) lupus syndrome. This means the baby may have a rash and/or a slow heartbeat. There's a slightly greater risk in following pregnancies, so make sure you discuss this in detail with your rheumatology and obstetric team before considering another pregnancy. Most babies born to mothers with anti-Ro antibodies will be fine, but it's important to have regular scans of the baby's heart during the pregnancy.
Monique was 25 and visiting family in the Caribbean, where she should have been having the time of her life.
Even though Monique was young, sociable and loved life, she wasn't enjoying the trip to the beautiful island where her parents grew up. In fact, she was depressed, had lost her appetite and her energy levels were low.
Her concerned family took her to a hospital to have tests carried out, but nothing was discovered.
One day later that holiday while Monique and her family were at the cinema, she became very unwell.
Monique said: "When the movie was finished, I couldn't put my feet on the ground, I was in excruciating pain. It was like pins and needles multiplied by 1,000 times.
"I had to be carried out of the cinema. It was scary."
This was back in 2007 and shortly after her return home to London, Monique went to her local hospital for further tests. This led to a diagnosis of lupus.
"I remember thinking, 'How can I get this? I'm young, fit and healthy'. It was a big adjustment for me and my family, to be my age and to be diagnosed with something as serious as lupus."
Monique has had some tough times since her diagnosis.
Monique developed lupus nephritis, which is an inflammation of the kidneys that can affect people with lupus. It's potentially a serious problem and Monique needed chemotherapy to treat it.
"Kidney involvement can happen really quickly," Monique said. "Chemotherapy isn't something to be taken lightly, but I couldn't consider for too long whether I would have it or not.
"It worked for me, but I know for others it hasn't been so successful."
Another difficulty that has affected Monique has been the fatigue brought on by her condition. She said: "I have to plan the week ahead. I can only really do two to three things a week.
"Fatigue is always there. However you structure your time, you have to do it around fatigue.
"Sometimes you don't have to be doing much and you get this overwhelming need to sit or lie down."
As well as planning her time carefully, a healthy lifestyle is important for Monique. She said: "I try to sleep and eat properly. I've cut out red meat and swapped it for fish, chicken and vegetables. I also drink lots of water."
While Monique has found these changes to be beneficial, she finds it hard to always keep on track with what she eats.
She said: "I'm literally having to change food habits of a lifetime, which isn't always easy, so that in itself can bring on depression as I think about 'my past life' compared to how I am now. It's all very much part of a grieving process, which doesn't have any timescale of a beginning or end."
Monique used to run the Enfield Lupus Support Group, and she has made great friends through the group in north London.
"The support group has been extremely beneficial to help with depression," she said.
"Everyone's symptoms are different. Sharing information can really help."
At the support group, members share experiences and problems with people who really understand. Meaningful practical and psychological advice is offered and there is real compassion, warmth and friendship.
A feeling of acceptance and belonging at what can be a scary and confusing time in people's lives, can make a world of difference.
Monique said: "Lupus has affected my social life. The condition can have that impact on social circles, particularly old social circles from school or something like that, with people who don't have lupus.
"Sometimes the thought of going out is really appealing, but then I think 'well, I'll have to have a bath, get dressed, get ready and then call a cab' and all that can seem like too much and it puts me off.
"Then I cancel and that can annoy people, and so people stop asking you to social events.
"I don't take anything for granted now. This condition has really taught me that."
You can read more of our supporter stories on our Your stories page.