Systemic lupus erythematosus

Published by Bupa's Health Information Team, October 2010.

This factsheet is for people with systemic lupus erythematosus, or who would like information about it.

Systemic lupus erythematosus, or lupus, is an example of an autoimmune disease whereby the immune system attacks some of the body's healthy tissues, causing inflammation and damage. Most people have a mild form of the condition.

About systemic lupus erythematosus

In the UK, one in 3,000 people has lupus and it's nine times more common in women than it is in men. The symptoms of lupus usually develop between your late teens and early 40s. You're more likely to be affected if you are of African, Caribbean or Asian decent.

Lupus is an autoimmune condition. Normally, your body's immune system produces white blood cells and proteins called antibodies to fight infection. If you have lupus, your immune system wrongly attacks healthy tissues, as it doesn't recognise them, and causes inflammation.

There are two main forms of lupus - systemic and cutaneous. Systemic lupus can affect nearly every tissue and organ system in your body. Cutaneous lupus only affects the skin (usually on your face, scalp or hands.) This factsheet will focus on the systemic form of lupus which is referred to as SLE.

Symptoms of systemic lupus erythematosus

There are a number of different symptoms of SLE. Most people generally feel tired and unwell, and can have a fever. Other common symptoms of SLE are:

• aching joints (arthralgia) and swollen joints (arthritis), particularly in your hands, wrists and knees
• a skin rash that may appear on your face and is often butterfly shaped
• weight change
• sun or light sensitivity (photosensitivity)
• hair loss (alopecia)
• ulcers (often in clusters) in your mouth or nose

Some people develop more serious problems because SLE can prevent major organs from functioning properly. The most common organ systems affected are listed below.

• Heart and lungs. About half of people with SLE have inflammation in the tissues lining their heart or lungs. This may cause sharp chest pains (which are often worse if you breathe in deeply or sit forward), coughs or breathlessness.
• Kidneys. Up to one in three people with SLE have inflamed kidneys, but this doesn't usually cause further problems. Some people, however, can develop a more serious form of kidney disease and will need to be monitored closely.
• Brain and nervous system. About one in three people with SLE are affected by migraine, which is treated in the same way as the common form. Rarely, inflammation can develop in nerves. This may lead to numbness or tingling in your face, arms or legs, foot drop (where you can't lift the front part of your foot), or visual or hearing problems. Some people may have fits (similar to epilepsy) or develop psychiatric symptoms such as depression, confusion or paranoia, but this is rare.

SLE can also affect your bone marrow and lead to anaemia. This makes you feel tired and your skin becomes pale.

The symptoms of SLE can range from mild to life-threatening. Although they may not necessarily be a result of SLE, you should see your GP if you have any of these symptoms.

Complications of systemic lupus erythematosus

If you have SLE, you may also have another autoimmune disease, such as antiphospholipid syndrome, Sjögren's syndrome or rheumatoid arthritis, as the conditions sometimes occur together.

Women with SLE have an increased risk of complications during pregnancy. These include miscarriage, pre-eclampsia, reduced growth, premature birth and stillbirth. If you are pregnant, or trying for a baby, talk to your GP or midwife about how these risks affect you. You will usually be monitored by your obstetrician (a doctor who specialises in pregnancy and childbirth) and your rheumatologist (a doctor who specialises in conditions affecting muscles and bones, particularly the joints and surrounding tissues).

Causes of systemic lupus erythematosus

The exact cause of SLE is unknown. It's thought, however, that a combination of environmental, genetic and hormonal factors are involved. You may be more likely to develop SLE, or make it worse, if you:

• are exposed to sunlight
• have been infected with the Epstein-Barr virus
• work in an environment where you are exposed to silica, pesticides or mercury

If you are a woman and have SLE, you may be advised not to take a contraceptive pill which contains high doses of the hormone oestrogen, as this may aggravate your condition. If you take hormone replacement therapy (HRT), this may also cause SLE to flare up. Ask your GP for advice before taking these medicines.

SLE can be sometimes be caused by certain medicines, such as minocycline, isoniazid, hydralazine, procainamide, chlorpromazine, sulfasalazine, losartan and anti-convulsants. However, this is usually only temporary. Once you stop taking the medicine, symptoms usually stop.

Diagnosis of systemic lupus erythematosus

Your GP will examine you and ask about your symptoms. He or she may take a blood sample to look for antibodies. Depending on the results of the blood test, your GP may refer you to a rheumatologist.

SLE is often difficult to diagnose as the symptoms can be similar to other conditions and may come and go. Therefore, further tests may be needed to see which of your organs have been affected. These may include:

• an X-ray
• an ultrasound scan, which uses high-frequency sound waves to produce an image of the inside of your body
• a CT (computerised tomography) scan, which uses X-rays to build up three-dimensional pictures of the inside of your body
• an MRI (magnetic resonance imaging) scan, which uses magnets and radio waves to produce two- and three-dimensional pictures of the inside of your body

Treatment of systemic lupus erythematosus

Most people with SLE can manage their condition with self-help measures and medicines. Although SLE can't be cured, it can often be limited and made much better (remission). Your rheumatologist will advise you which treatments are most suitable for you.

Self-help

To help reduce the symptoms of SLE:

• don't sunbathe
• use high-factor sunblocks or sunscreens (factor 25 and over), a hat and clothing to protect your skin from the sun as much as possible
• try to do low-impact exercises such as walking and swimming, but rest when you need to

Medicines

NSAIDs
If you have muscle and joint pain you can take over-the-counter non-steroidal anti-inflammatory medicines (NSAIDs) such as ibuprofen. Long-term use of some NSAIDs isn't recommended as they can cause potentially serious complications such as stomach ulcers, high blood pressure and kidney problems. NSAIDs may cause reduced fertility in women, but this is reversed as soon as treatment is stopped. Always read the patient information leaflet that comes with your medicine and if you have any questions, ask your pharmacist for advice.

Antimalarials
Your rheumatologist may prescribe antimalarials such as hydroxychloroquine or chloroquine to help milder symptoms such as joint pain, tiredness and skin rashes. Side-effects include stomach and bowel upsets and headaches. As these drugs can potentially cause eye problems, you will need to have regular eye checkups with an optician or ophthalmologist (a doctor who specialises in eye health, including eye surgery).

Steroids and immunosuppressants
For milder symptoms such as skin rashes, your GP may prescribe a steroid cream. If you have more serious symptoms, such as inflammation in the lining of your heart or lungs, you will be given steroid tablets. Higher steroid doses may be needed if you have inflamed kidneys or severe anaemia. The possible side-effects include weight gain and muscle weakness.

If you need to take steroids, you will usually be given immunosuppressive medicines at the same time. These work by suppressing your immune system so it doesn't attack your body's tissues; they also prevent long-term complications of steroid use such as osteoporosis. You will need to have regular blood tests to monitor your liver and kidney functions.

Your GP may also recommend you have vaccines for influenza (flu), Haemophilus influenzae type B (Hib) and pneumococcus (which can cause pneumonia and meningitis). This will help to reduce your risk of becoming infected with such disease-causing organisms.

For answers to frequently asked questions on this topic, see Common questions.

For sources and links to further information, see Resources.