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Systemic lupus erythematosus

 
Systemic lupus erythematosus (SLE or lupus) is a 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 (most commonly the skin, muscles, joints) causing inflammation and tissue damage.
 
Common lupus symptoms include fatigue, fever, muscle aches and facial rash.  There is no cure for lupus so the aim of treatment is to relieve symptoms and protect body systems and organs.

Causes 

Anyone can develop lupus but it is around eight times more common in women than men. It can develop at any age but most frequently between the ages of 20 and 45 years.  In New Zealand, lupus is more prevalent in Maori, Pacific Island 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 lupus 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 lupus.

The causes of lupus 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 lupus 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 lupus, and because some women experience worsening symptoms at the time of their menstural periods, 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, rheumatoid arthritis, high blood pressure and heart conditions, can lead to the development of lupus 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.

Signs and symptoms

There is great individual variation as to the nature and severity of lupus 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.   Lupus 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 lupus 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
  • Loss of appetite
  • Weight loss
  • Skin rashes - most commonly a characteristic rash across the nose and cheeks known as the "butterfly rach" or "malar rash"
  • Hair loss
  • Mouth and nose ulcers
  • Red sore dry eyes
Body systems and organs sometimes affected by lupus 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 lupus may experience nausea, vomiting, diarrhoea or abdominal pain due to inflammation of the structures and organs within the abdomen.

Nervous system
Lupus 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 lupus 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 lupus 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).

Other conditions can occur at the same time as lupus, including fibromyalgia, coronary heart disease, pancreatitis and infections.
 
Systemic lupus erythematosus should not be confused with discoid lupus erythematosus. This is a similar condition but only affects the skin. It's 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 systemic lupus erythematosus.

Diagnosis 

There is no single test to diagnose lupus. 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 lupus. 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.  Lower than normal white and red blood cell counts are associated with lupus

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)
This is a measure of red blood cells (erythrocytes) settling in a test tube over a given period of time.An elevated ESR indicates the presence of inflammation in the body.
 
C-reactive protein
An elevated level of this protein in the blood 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 lupus 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, lupus is unlikely.

Other autoantibody tests
Sometimes tests for other autoantibodies that are specific to lupus 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 lupus.
If lupus is suspected or confirmed a referral to a specialist (usually a rheumatologist) may be recommended.

Treatment 

While lupus 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 lupus can look forward to a normal life span.   Treatment options include:

Medications
Anti-inflammatory medications such as aspirin, and non-steroidal anti-inflammatory drugs (NSAID's) such as Brufen and Voltaren, can help to reduce pain and inflammation. To avoid irritating the stomach, it is recommended that these medications be taken with food or with a stomach protectant such as misoprostol (Cytotec).  Other medications that may be used include:
 
Corticosteroid medications - These are commonly used in the treatment of moderate to severe cases of lupus 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 lupus are prednisone (Apo-Prednisone), hydrocortisone (Locoid) and dexamethasone (Hospira).

Anti-malarial medications - It is not entirely clear how these medications work to treat lupus, however it is thought that they reduce the autoimmune response. An anti-malarial medication commonly used in the treatment of lupus 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. Immunosuppressive medications are used for treating people with more severe disease, such as when there is damage to internal organs.  Examples of these medications include azathiaprine (Imuprine), methotrexate (Methoblastin), cyclophosphamide (Cycloblastin) and mycophenolate mofeti (Cellcept) which is particularly useful for people with kidney disease.

Biological medications - These are synthetic (man-made) antibodies that suppress specific components of immune system function.  They include rituximab (Mabthera) which is also helpful for people with kidney disease, and belimumab (Benyista).
 
Gamma globulins - In severe cases of lupus 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 lupus is more problematic, more rest is required.

Avoiding sunlight
Because sunlight can trigger flares and skin rashes in some people with lupus, it is important that lupus 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 lupus 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 lupus 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 dietician.

Further support

For further information and support (including services available in your area) contact:
 
Arthritis New Zealand:
Ph: 0800 663 463
Website: www.arthritis.org.nz 

Lupus Trust of New Zealand
Ph: 0800 324 634
Website: www.lupus.org.nz  
 
Lupus Care and Support Inc.
Ph: 021 0286 1056
Website: www.lupussupport.org.nz  

References 

Arthritis New Zealand (date unknown) Lupus. Pamphlet. Wellington: Arthritis New Zealand www.arthritis.org.nz/wp-content/uploads/2011/08/Lupus-DLE-Brochure-LR.pdf
Lupus Trust of New Zealand (2013) What is lupus? Auckland: Lupus Trust of New Zealand - www.lupus.org.nz/whatis_lupus.php
O’Toole, MT (Ed.). (2013) Systemic lupus erythematous (SLE). Mosby’s Dictionary of Medicine, Nursing & Health Professions (9th ed.). St Louis: Elsevier Mosby.
Shiel, WC (2012) Systemic lupus erythematosus (SLE or Lupus). MedicineNet.com. New York: WebMD LLC.
 
Last Reviewed – June 2013
 
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