Systemic Lupus Erythematosus
Systemic lupus erythematosus (SLE) is a chronic (long-term) autoimmune disease, where the body’s immune system attacks and damages its own healthy tissue. It can affect any part of the body (but most commonly the skin, muscles, joints) causing inflammation and tissue damage.
Anyone can develop SLE, but it is around nine times more common in women than men. It can develop at any age but most frequently between the ages of 15 and 45 years. In New Zealand SLE is more prevalent in Maori, PacificIsland and Asian races than Europeans. It’s usually a mild disease but can be severe in around 5% of cases.
A healthy immune system produces proteins called antibodies to fight and destroy infectious agents such as bacteria and viruses. However, in SLE the immune system produces “autoantibodies” that attack the body's own healthy tissues. It is not known why autoantibodies form but they cause much of the tissue damage and inflammation associated with SLE.
The causes of SLE are unknown. It is thought that a combination of factors may work together to trigger the development of the condition, including:
Genetic (inherited) factors
Although uncommon, the tendency to develop the condition may run in families giving rise to the theory that genetic factors influence its development.
Infectious agents
Some research has indicated that SLE is triggered by the abnormal response by the body’s immune system to a viral infection.
Hormonal factors
Because substantially more women than men suffer from SLE, female hormones are thought to play a role in the development of the disease.
It is known that some medications, such as those used for treating epilepsy, high blood pressure and heart conditions, can lead to the development of SLE symptoms. When this occurs it is referred to as drug induced lupus. This is a rare occurrence, however, and symptoms disappear when the medications are stopped.
There is great individual variation as to the nature and severity of SLE symptoms, from a mild condition that affects only one area of the body to a rapidly progressive disease that can affect many areas of the body at once. Fortunately it is usually a mild disease that most commonly affects the skin, muscles and joints.
SLE is characterised by periods of remission (absence of symptoms) and "flares" (when symptoms are problematic). Flares can be triggered by factors such as sunlight, increased stress and some medications.
The first symptoms of SLE may be vague. There may be a feeling of being generally unwell or of having a continuous dose of the flu. Muscle and joint aches, and fever may also be present.
Subsequent symptoms may include:
- Fatigue
- Weakness
- Weight loss
- Skin rashes - most commonly a characteristic rash across the nose and cheeks known as the "butterfly" or "malar" rash
- Hair loss
- Mouth ulcers
- Red sore dry eyes
Body systems and organs sometimes affected by SLE include:
Heart and lungs
Inflammation of the outer lining of the lungs (pleurisy) may cause chest pain and shortness of breath. Inflammation of the outer or inner lining of the heart (pericarditis, endocarditis) may also cause chest pain and lead to other problems such as heart rhythm disturbances, damage to the heart muscle and heart failure. The arteries that supply blood to the heart may also become inflamed (coronary vasculitis), affecting the amount of blood able to reach the heart muscle. Again this may produce symptoms such as chest pain (angina) or shortness of breath.
Kidneys
Inflammation can damage the delicate filtering structures within the kidneys. This can reduce their ability to filter the blood and can lead to alteration in kidney function, and in some cases, kidney failure.
Gastrointestinal tract
Some people with SLE may experience nausea, vomiting, diarrhoea or abdominal pain due to inflammation of the structures and organs within the abdomen.
Nervous system
SLE can affect the brain or central nervous system in some people causing headaches, seizures, vision problems and dizziness. Personality changes and depression can also occur.
Blood and blood vessels
People with SLE may develop anaemia (lack of oxygen carrying haemoglobin in the blood) or leucopaenia (a decreased number of infection fighting white blood cells). These conditions often compound the feeling of fatigue and ill health. Inflammation of the blood vessels (vasculitis) may also occur and some people with SLE may have an increased risk of blood clots.
Musculoskeletal system
Arthritic pain, stiffness and swelling of the joints can occur - especially in the small joints of the hands and feet. Muscle aches are common and distortion of the hands due to inflammation in the tendons occurs in approximately 20% of cases. There may also be an increased tendency to develop osteoporosis (loss of bone mass).
SLE should not be confused with discoid lupus erythematosus. This is a similar condition to SLE, but only affects the skin. The condition is characterised by a raised, coin-shaped red rash on the skin, which may become thickened and scaly. A small percentage of people with discoid lupus erythematosus will go on to develop SLE.
There is no single test to diagnose SLE. It is a complex disorder and it can be difficult to diagnose. To assist with a diagnosis the doctor will take a full medical history, including any family history of SLE. They will discuss nature and severity of current symptoms experienced and will conduct a thorough physical assessment. A urine sample will also be analysed to check for kidney involvement.
In order to confirm a diagnosis, blood tests will be taken. Blood tests performed may include:
Complete blood count
This will show the number of red and white blood cells.
Biochemistry
This will show the balance of electrolytes in the body and can indicate abnormalities with the function of the kidneys and other internal organs.
Erythrocyte sedimentation rate (ESR)
An elevated ESR indicates the presence of inflammation in the body.
Antinuclear antibody test (ANA)
The ANA test detects the presence of autoantibodies. This test is positive in 95% of SLE cases, however there are a number of other causes for a positive ANA result, including other autoimmune diseases and infection. If the test is negative, SLE is unlikely.
Other autoantibody tests
Sometimes tests for other autoantibodies that are specific to SLE are performed to confirm a diagnosis. These may include tests for complement enzymes, antiphosphiloid antibodies, lupus anticoagulant and antibodies to DNA (anti-dsDNA).
Coagulation studies
These measure the clotting ability of the blood. Abnormalities can be associated with an increased risk of blood clots in some people with SLE.
If SLE is suspected or confirmed a referral to a specialist (usually a rheumatologist) may be recommended.
While SLE cannot be cured, it can be controlled. Treatment involves monitoring the condition, preventing flares and treating the symptoms when they occur. The type of treatment given will depend on the severity of the condition and the degree to which the different body systems are affected. Often treatment is a team approach with several different health professionals involved and a combination of treatments used. With appropriate treatment 80%-90% of people with SLE can look forward to a normal life span.
Treatment options include:
Medications
Anti-inflammatory medications (such as aspirin) or non-steroidal anti-inflammatory drugs (NSAID's) can help to reduce pain and inflammation. To avoid irritating the stomach, it is usually recommended that these medications be taken with food.
Other medications that may be used include:
Corticosteroid (steroid) medications - These are commonly used in the treatment of moderate to severe cases of SLE and work by suppressing inflammation. They can be given as tablets, as a cream applied to the skin, or by injection. Examples of these medications used to treat SLE are prednisone, hydrocortisone and dexamethasone.
Anti-malarial medications - It is not entirely clear how these medications work to treat SLE, however it is thought that they reduce the autoimmune response. An anti-malarial medication commonly used in the treatment of SLE is Hydroxychloroquine.
Immunosupressive medications - These help to dampen down the abnormal response of the immune system and can be given as tablets or as an infusion into a vein. Examples of these medications used to treat SLE include azothiaprine, methotrexate and cyclophosphamide.
Gamma globulins - In severe cases of SLE these medications may be given to strengthen the immune system.
Rest and Exercise
Rest will help reduce fatigue, pain and inflammation. Exercise is also important to maintain muscle strength, decrease joint deformities and stiffness, and to maintain mobility. A physiotherapist can recommend an appropriate exercise regime. When SLE is more problematic, more rest is required.
Avoiding sunlight
Because sunlight can trigger flares and skin rashes in some people with SLE, it is important that SLE sufferers who are sensitive to the sun should avoid sun exposure, particularly between 11.00am and 3.00pm. Protective clothing and sunscreens with a sun protection factor of 15+ should be worn when in the sun.
Alternative therapies
Reducing stress is vital for people with SLE and many people find that techniques such as meditation, yoga and hypnotherapy can assist with stress reduction.
Acupuncture and massage is effective for some people in reducing joint and muscle pain.
Dietary factors are also important and a diet low in red meat and dairy products is thought to help reduce pain and inflammation. Also, some foods, such as alfalfa sprouts, and dietary supplements, such as Echinacea, have been implicated in causing SLE flares and should be avoided.
Homeopathy and vitamin supplements may also assist with overall improved health.
It is important to discuss any alternative therapies with the doctor or specialist before undertaking them to insure that they will not interfere with medical treatments. For advice on dietary factors, consult a dietitian.
For further information and support please contact Arthritis New Zealand:
Arthritis New Zealand
PO Box 10-020
Wellington
Ph: (04) 472 1427
Fax: (04) 472 7066
Website: www.arthritis.org.nz
For branch offices please refer to the local telephone directory or Arthritis New Zealand website.
Lupus support groups include:
Anderson, K.N., Anderson, L.E. & Glanze, W.D. (eds.) ( 2006) Mosby’s medical, nursing, & allied health dictionary (6th ed.) St. Louis: Mosby-Year Book, Inc.
Arthritis New Zealand (2007) Lupus. Pamphlet. Wellington: Arthritis New Zealand
Carson-De Witt, R. (2006) Systemic lupus erythematosus. The Gale Encyclopedia of Medicine. Third Edition. Jacqueline L. Longe, Editor. Farmington Hills, MI. Thompson Gale.
Gill, M. (ed.) (2000) A handbook for the interpretation of laboratory tests Auckland: Diagnostic Medlab Ltd.
Lupus Trust of New Zealand (2004) What is lupus? Auckland: Lupus Trust of New Zealand - http://www.lupus.org.nz/whatismore.html
Last Reviewed – 5/09/07
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