Systemic lupus erythematosus (SLE) can cause various symptoms, the most common being joint pains, skin rashes and tiredness. Problems with kidneys and other organs can occur in severe cases. Treatment includes anti-inflammatory painkillers to ease joint pains. Steroids and/or other medication are sometimes also needed.
Systemic lupus erythematosus (SLE) is a chronic (persistent) disease that causes inflammation in various parts of the body. It is commonly just called SLE or 'lupus'. The severity of SLE ranges from mild to severe. There are two main forms of lupus. Discoid lupus only affects the skin and is not discussed in this leaflet. The other form is SLE, which involves the skin and joints and may involve internal organs such as the heart or kidney as well.
SLE affects about 5 in 10,000 people in the UK. It is ten times more common in women than in men. It most typically develops in women aged between 20 and 40. However, anyone at any age can be affected. It is more common in people from Afro-Caribbean, Asian, or Chinese origin. Although SLE can run in families, only 3 in 100 children of people with SLE will actually develop the disease.
SLE is an autoimmune disease. This means that the immune system (which normally protects the body from infections) mistakenly attacks itself. This can cause symptoms and may damage the affected parts of the body. Other autoimmune diseases include diabetes, rheumatoid arthritis and thyroid disorders.
It is not known why SLE occurs. Some factor may trigger the immune system to attack itself. Possible triggers of SLE include infections, drugs (for example, minocycline or hydralazine) or sunlight. Hormonal changes may play a role in SLE, which could explain why it is much more common in women.
The symptoms and severity of SLE vary tremendously between people. Many people have fatigue (tiredness), weight loss and a mild fever. In addition, one or more of the following may develop.
Most people with SLE develop some joint and muscle pains. Sometimes only a few joints are affected, whereas other people have many joints affected. The small joints of the hands and feet tend to be the ones affected most. The pains may 'flit' from joint to joint. Joint stiffness is common and is usually worse first thing in the morning. Mild joint swelling may occur but severe arthritis with joint damage is unusual.
A red rash which develops over the cheeks and nose is common (the 'butterfly rash'). Other areas of skin exposed to sunlight (hands, wrists, etc) may also develop a rash. About 6 in 10 people with SLE find that their skin is very sensitive to sunlight. Various other rashes may develop. The blood vessels just under the skin may also be affected and cause poor circulation to the fingers and toes (Raynaud's phenomenon). Mouth ulcers are more common in people with SLE. Some hair may fall out (alopecia). Any hair loss tends to be minor and cause hair 'thinning' rather than bald patches. However, quite serious hair loss sometimes develops, although the hair often grows back when SLE is less active.
A mild anaemia is common. Other blood problems, such as reduced numbers of white blood cells or platelets (the cells that help the blood to clot), are less common. A tendency to form blood clots is an uncommon complication. Some lymph glands may swell.
The tissues that cover the heart and lung (the pleura and pericardium) may become inflamed. This can cause pleurisy (pains in the side of the chest) or pericarditis (central chest pains). The actual heart or lung tissue is less commonly affected.
Around 1 in 3 people with SLE may develop inflammation of the kidneys, which can lead to the kidneys leaking protein and blood into the urine. This does not usually cause problems unless the disease is very severe. Kidney failure is an uncommon complication.
Mental health problems in SLE are fairly common and include depression and anxiety. Although mild depression can be part of the disease itself, it can also be due to your reaction to having a serious illness. It is not uncommon for people to have difficulties in coping with having SLE. It is important to share any feelings you have with your doctor, as treatment can be really beneficial. Occasionally, inflammation of the brain can lead to epilepsy, headaches, migraines and other conditions.
In some cases, the symptoms develop quite slowly. At first they may be confused with other problems, as there are many possible causes of joint pains and tiredness. Sometimes several symptoms occur together. Symptoms range from mild to severe. For example:
Typically, there are times when the disease flares up (relapses) and symptoms become worse for a few weeks, sometimes longer. These relapses tend to alternate with times when symptoms settle down (remission). The reason why symptoms flare up or settle down is not yet fully understood.
If your symptoms suggest SLE then your doctor will usually do some blood tests. Most people with SLE have an antibody called antinuclear antibody in their blood. (Antibodies are small proteins that are part of the immune system.) Another antibody called anti-double-stranded DNA (anti-dsDNA) is a often present in people with SLE. Various other antibodies are also associated with SLE. However, they can also occur in perfectly well people who do not have SLE. Typical symptoms combined with high levels of certain antibodies usually indicate that you have developed SLE.
Once SLE is diagnosed, you will normally be advised to have regular checks and tests. For example, regular blood tests to check for anaemia and urine tests to check for kidney problems. A blood test to measure a blood chemical called 'complement' (another part of the immune system) can assess the activity of the disease. The level of this chemical reflects how 'active' the disease is.
Other tests including scans and X-rays may be advised to check on the function of the heart, kidneys and other organs if the disease is thought to be affecting these areas of the body.
Although there is no cure for SLE, this condition can usually be controlled and symptoms eased. Most people with SLE are seen regularly by a specialist who advises on treatment. The treatments may vary from time to time, depending on the severity of the disease or flare-up of symptoms and also which parts of the body are affected. You may even not need any treatment if you have very mild symptoms.
Treatment options include the following:
These are often called anti-inflammatory painkillers and are commonly prescribed to ease joint or muscle pains. Examples of these are ibuprofen, naproxen and diclofenac. The main possible side-effects from NSAIDs are stomach and gut problems such as pain or bleeding in the stomach. If necessary, other medication can be prescribed to protect the stomach from these possible side-effects.
Hydroxychloroquine is often effective at improving skin problems, tiredness and joint pains that are not well controlled by NSAIDs. It is not clear how this drug works in SLE. It may take 6-12 weeks for it to become fully effective. The dose is often reduced to a lower 'maintenance' dose once symptoms have eased. Many people with SLE take this drug long-term to keep symptoms away. Side-effects are uncommon. The most serious is damage to the eye, which is unusual. Your doctor is likely to check your vision before you start it and then every year. If you are taking this drug and notice any changes in your vision, you should inform your doctor promptly.
Steroid tablets are usually advised if you develop more severe symptoms. Steroids reduce inflammation and the dose is usually given at the lowest possible dose, in order to reduce any side-effects from the steroids. Steroids may cause side-effects if taken for long periods. These include thinning of the bones (osteoporosis), thinning of the skin, weight gain, muscle wasting, high blood pressure and other problems.
Drugs such as azathioprine, ciclosporin, cyclophosphamide, methotrexate and mycophenolate may be advised if you have severe SLE. These drugs are called immunosuppressive drugs because they work by suppressing the immune system. One side-effect of these drugs is that you will be more prone to developing infections. If you take one of these, you need to have regular blood and urine tests to look out for possible side-effects.
Most people with SLE lead active, normal lives. The outlook for people with SLE is much better than it was in the past. Modern treatments are more effective. For many people with SLE, symptoms are mild or moderate with little risk to life. The joint and skin symptoms may persist, but can usually be eased with treatment. For most people with SLE, the pattern of their disease becomes established within ten years; so, if serious problems have not developed in this time then they are unlikely to do so.
For a few people, SLE is severe and can be life-threatening. Severe inflammation of the kidneys, leading to kidney failure, can rarely occur. Severe brain involvement is also rare but can be very serious. However, modern immunosuppressive treatments have improved the outlook, even for people with severe disease. Some people find that symptoms settle in their middle age and they can come off all treatment.
This leaflet is only an introduction to a disease that can be confusing. Further help, support and information can be obtained from:
St James House, Eastern Road, Romford, Essex, RM1 3NH
Tel: 01708 731251 Web: www.lupusuk.com
Copeman House, St Mary's Court, St Mary's Gate, Chesterfield, Derbyshire, S41 7TD
Tel: 0300 790 0400 Web: www.arthritisresearchuk.org