Lupus erythematosus is a chronic inflammatory disease that occurs when your body’s immune system attacks your own tissues and organs. Symptoms range from mild to life-threatening
There are some types of lupus that just affect the skin – such as discoid lupus erythematosus and subacute cutaneous lupus erythematosus. Some medications can also cause lupus-like side effects.
However, the term “lupus” is most often used to describe a more severe form of the condition called systemic lupus erythematosus (SLE), which can affect many parts of the body, including the skin, joints and internal organs.
Symptoms range from mild to severe, and many people will have long periods with few or no symptoms before experiencing a sudden flare-up, where their symptoms are particularly severe.
Even mild cases can be distressing and have a considerable impact on a person’s quality of life.
The history of lupus begins in 1828 when the French dermatologist, Biett described the disease. For the next 45 years, studies of the disease showed nothing more than descriptions that emphasized skin changes. In the mid 1800’s, Pierre Cazenave was the first person to have a comprehensive description of lupus. The disease was named because of a wolf-bite shaped rash (the butterfly rash) that appears across the nose and cheeks of many lupus patients. “Lupus” is the Latin word for wolf.
In 1873, a dermatologist named Kaposi noted that people with a history of lupus lesions also experienced problems in internal organs. Then in the 1890’s, a famous American physician, Sir William Osler, discovered that some patients had internal organ involvement but no history of lupus skin problems at all. In 1948, a finding by Dr. Malcolm Hargraves of the Mayo Clinic that showed that patients with SLE had a LE cell in their blood, allowed doctors to develop a simple blood test. This blood test, along with a medical history and family history of lupus was used to diagnose many more cases of lupus. In the 1950’s, scientists discovered antinuclear antibodies (proteins that cause the immune system to attack its own tissues) which lead to the development of more sensitive tests for SLE. Studies using mice (murine models) in the last 40 years have also increased our understanding of the disease.
Now, after decades of little action, things are happening. The community is seeing unprecedented interest from biotechnology and pharmaceutical companies that translates into dozens of companies interested in lupus and even more trials for lupus patients. (source: https://lupusincolor.com/history-2/)
This page focuses on SLE.
Symptoms of Lupus
Symptoms of systemic lupus erythematosus (SLE) can vary widely from person to person. Some people may only experience a few mild symptoms, whereas others may be more severely affected.
Even if you usually have mild symptoms, SLE can “flare-up”, with symptoms becoming more severe or new symptoms developing.
The three main symptoms of SLE are fatigue, joint pain and rashes.
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 housework or office work, can leave you feeling exhausted.
Many people with SLE find that fatigue is the most distressing and disruptive aspect of SLE because it has a negative impact on their work and social life.
If you have SLE, you are 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, and is usually worse in the morning.
Unlike some other conditions that affect the joints, SLE is unlikely to cause your joints to become permanently damaged or deformed.
Many people with SLE develop rashes on their skin – most commonly 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” or “malar rash”.
Rashes caused by SLE may get better after a few days or weeks, but can last longer or even be permanent.
Rashes caused by SLE can sometimes be itchy or painful, and they may get worse if they are exposed to sunlight. This is known as “photosensitivity”.
SLE can also cause a wide range of symptoms. However, you’re unlikely to have all of the symptoms listed below, and many people with the condition will only experience 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
- 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)
When to seek medical advice
You should see your GP if you have persistent or troublesome symptoms that you think could be caused by SLE.
While it is likely that your symptoms are being caused by a more common condition, it is important to see a doctor for a diagnosis.
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 body’s natural defence against illness and infection. When the immune system detects the presence of an infectious agent, such as bacteria or a virus, it sends white blood cells and antibodies to attack it.
In cases of SLE, antibodies released by the immune system can attack healthy tissue, cells and organs.
It’s not clear exactly why this happens, although most experts think SLE has more than one cause. It has been suggested that there may be a number of genetic factors that make people more susceptible to the condition, and that it also takes one or more environmental factors to trigger SLE in those who are susceptible.
Possible genetic and environmental factors are discussed in more detail below.
Brothers and sisters of people with SLE are much more likely to develop the condition than the population at large.
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 body’s processes not working normally.
Most faulty 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.
A number of environmental factors may be responsible for triggering SLE in vulnerable individuals, although the evidence for many of these is limited.
Possible environmental factors that have been suggested include:
- exposure to sunlight (ultraviolet light)
- hormonal changes that occur during a woman’s lifetime, such as during puberty or pregnancy
- certain infections, such as by the Epstein-Barr virus (EBV) – a common viral infection that doesn’t usually cause any symptoms
Systemic lupus erythematosus (SLE) can be difficult to diagnose, as it has similar symptoms to several other, far more common, conditions.
Diagnosis may also be difficult because symptoms can vary greatly from person to person, and they may change over time. For example, there may be periods where your symptoms are not very noticeable, or times when they flare up and become more severe.
For a confident diagnosis of SLE to be made, you will need to have several symptoms of lupus and a number of blood tests may need to be carried out.
Some of the blood tests that may be carried out are described below.
Erythrocyte sedimentation rate (ESR) test
A blood test called the erythrocyte sedimentation rate (ESR) test can be used to determine whether there is any inflammation in your body.
This can be useful in diagnosing SLE because the condition can cause many areas of the body to become inflamed (swell), including the joints and internal organs.
The test works by measuring 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 there are high levels of inflammation.
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. Other blood tests will also be needed to confirm the diagnosis.
Anti-DNA antibody test
An anti-DNA test also checks for a certain type of antibody in your blood, known as the anti-DNA antibody. If you have the anti-DNA antibody, it is highly likely that you have SLE. However, the antibody is only found in around 70% of people with 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. The level of this chemical may be tested to check how active your SLE is. The level of complement in your blood decreases when SLE is more active.
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 kidney problems. Monitoring your condition will allow your doctor to check for these complications and, if necessary, treat them as soon as possible.
There is currently no cure for systemic lupus erythematosus (SLE), but treatments that can ease the symptoms and make it easier to live with are available.
In most cases, treatment will involve a combination of self-care measures and medication.
Protecting yourself from the sun
Exposure to sunlight can sometimes make symptoms such as rashes worse, and it’s important to protect your skin when in the sun.
This means wearing clothing that covers your skin, a wide-brimmed hat and sunglasses. You will also need to apply sunscreen with a high SPF to prevent sunburn. However, some people with lupus are not sun-sensitive and do not need to take extra precautions.
As people get most of their vitamin D as a result of direct sunlight on the skin, there is a risk you may not get enough of this vitamin if you need to avoid sun exposure. This means you may need to make an extra effort to include good sources of vitamin D in your diet to avoid problems such as osteoporosis (weakened bones), and you may be advised to take vitamin D supplements.
Non-steroidal anti-inflammatory drugs (NSAIDs)
Non-steroidal anti-inflammatory drugs (NSAIDs) are a common 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.
Commonly prescribed NSAIDs for SLE include ibuprofen, naproxen and diclofenac.
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. 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 you to check for any problems, and you may be prescribed an additional medication called a proton pump inhibitor (PPI) to protect your stomach.
Hydroxychloroquine is a medicine that has been used to treat malaria, but is also effective in treating some of the symptoms of SLE, 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 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 uncommon, but may include indigestion, diarrhoea, headaches and rashes.
Hydroxychloroquine may also cause more serious side effects in a small number of people. For example, in rare cases, this medicine can cause eye damage. 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.
These are a type of medicine that help reduce inflammation quickly. They can be very effective in treating symptoms of SLE, but are usually only prescribed if the condition is severe.
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:
- 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 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 azathioprine, methotrexate, mycophenolate mofetil and cyclophosphamide.
Immunosuppressants are sometimes used in conjunction with corticosteroids (see above) because these medicines may ease your symptoms more effectively when used together. Alternatively, the use of immunosuppressant medication may allow your corticosteroid dose to be reduced.
Immunosuppressant medication is usually only prescribed if you have severe SLE. This is because this type of medication is powerful and can cause side effects such as:
- loss of appetite
- swollen gums
- bruising or bleeding more easily
- 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 they are taken during pregnancy, 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.
Tell your GP if a side effect becomes particularly troublesome, as it may mean your dose needs to be adjusted.
Taking immunosuppressant medication can increase your risk of developing an infection. This is a particularly serious concern for people with SLE, because the organ damage that can occur as a result of the condition means infections are more likely to be life-threatening.
It is therefore very important to report any symptoms of a possible infection to your GP immediately.
Symptoms of infection may sometimes be similar to a flare up of SLE and include:
- a fever (high temperature)
- a cough or breathlessness
- burning when passing urine or passing blood in your urine
You should also try to 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 is a new type of medication used in people with severe SLE that doesn’t respond to other treatments.
Rituximab was originally designed to treat certain types of cancer, such as lymphoma, but 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 cells called “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.
Rituximab is not currently licensed for treating SLE in the UK, but your specialist may consider it an appropriate treatment for you. If your doctor suggests using rituximab, they should tell you that there are currently some uncertainties about how effective or safe it is in treating SLE.
Common side effects of rituximab include:
- flu-like symptoms, such as chills and a high temperature
In rare cases, rituximab can cause a more serious allergy-like reaction. Most reactions occur during or shortly after the treatment is given, so you will be closely monitored once your treatment begins.
Belimumab is a new medication given to people with active SLE who don’t respond to other treatments.
It works by binding to growth factors that are needed for the survival of B-cells. It is given directly into your vein over several hours, known as an infusion. The first three doses are given 14 days apart, and the medication is usually given once a month thereafter.
Belimumab is licensed for treating SLE in the UK, and there may be instances where your specialist may consider it an appropriate treatment for you. If your doctor suggests using belimumab, they should tell you that there are currently some uncertainties about how effective or safe it is in treating SLE.
Common side effects of belimumab include:
- flu-like symptoms, such as chills and a high temperature
- nausea and diarrhoea
- difficulty sleeping (insomnia)
- a cough, sore throat and blocked or runny nose
- joint pain
- increased risk of infections
- changes in blood pressure
In rare cases, belimumab can cause a more serious allergy-like reaction. Most reactions occur during or shortly after the treatment is given, so you will be closely monitored once your treatment begins.
Expert Patients Programme
Many people with SLE have reported that they found joining the Expert Patient Programme (EPP) very helpful.
This 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:
- increasing their confidence
- improving their quality of life
- helping them manage their condition more effectively
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 that you do not have any complications.
However, for some people, SLE can be a more serious condition that can cause life-threatening complications. Some of these complications are outlined below.
Around one in every three people with SLE will develop a potentially serious kidney disease called lupus nephritis, which is caused by prolonged inflammation of the kidneys.
Lupus nephritis tends to develop relatively early in the course of SLE, usually within five years of diagnosis.
Symptoms of lupus nephritis can include:
In many cases, lupus nephritis does not cause any noticeable symptoms. However, this does not mean the condition is not dangerous, as the kidneys could still be being damaged.
If you have SLE, it is likely you will need to have 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 mycophenolate mofetil or cyclophosphamide.
In a small number of cases, the kidney damage can become severe enough to require treatment with dialysis (where a machine is used to replicates many of the kidneys’ functions) or a kidney transplant.
Cardiovascular disease (CVD) is a general term for any type of health condition that affects the heart and arteries. It is often associated with blood clots and atherosclerosis (hardening and narrowing of the arteries).
People with SLE are more likely to develop CVD than the general population, because SLE can cause your heart and arteries to become inflamed and damaged.
If you have SLE, you can reduce your risk of CVD by making health lifestyle chances, 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 – at least 150 minutes (two-and-a-half hours) a week of exercise strenuous enough to leave you slightly out of breath is recommended
- cutting down on your alcohol consumption
SLE does not usually affect fertility, but it can increase your risk of experiencing pregnancy complications, such as:
- pre-eclampsia (a condition that causes high blood pressure during pregnancy)
- premature delivery
Some children of women with SLE can also be born with heart block (where 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 is best to plan this carefully with your doctors if possible. The risk of complications is higher if you become pregnant during periods where 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, you will need to be monitored closely by your specialist and by an obstetrician, so they can check for any problems.
Lupus UK is the only national registered charity supporting people with systemic lupus and discoid lupus and assisting those approaching diagnosis.