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Systemic lupus erythematosus (lupus)

 
Systemic lupus erythematosus (SLE or lupus) is a long-term autoimmune disease, where the body’s immune system attacks its own healthy tissue. Lupus can affect many parts 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.
 
In New Zealand, lupus may affect around one in 900 people, and is more prevalent in Māori, Pasifika, and Asians than in Caucasians. Anyone can develop lupus but it is around ten times more common in women than men. It can develop at any age but does so most frequently between the ages of 20 and 45 years. 

Causes

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 - There is a clear genetic component in lupus, with a person being at higher risk of developing the condition if they have a brother or sister with lupus.
  • Infectious agents - Some research has indicated that lupus is triggered by an 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 menstrual periods or pregnancy, female hormones are thought to play a role in the development of the disease. 
  • Vitamin D deficiency - Vitamin D is involved in immune system function and a lack of it has been linked to autoimmune diseases including lupus, Crohn's disease, and ulcerative colitis
  • Certain medications - 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. Medication-induced lupus is rare and symptoms disappear when the medications are stopped.

Signs and symptoms

The nature and severity of lupus can vary from a mild symptoms that affects only one area of the body to a rapidly progressive disease that can affect many areas of the body at once, including organs and systems. 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 having a 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 rash" 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 may cause chest pain and shortness of breath, while inflammation of the outer or inner lining of the heart can 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, affecting the amount of blood able to reach the heart muscle causing 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 have a reduced 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 some 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. 

Diagnosis 

There is no single test to diagnose lupus. It is a complex disorder and it can be difficult to diagnose. 

Your doctor will take a full medical history, including any family history of lupus. They will discuss the nature and severity of your symptoms and conduct a physical assessment. A urine sample will also be analysed to check for kidney involvement. To confirm a diagnosis, blood tests will be taken.

Blood tests performed may include:

  • Complete blood count to show the number of red and white blood cells (lower than normal white and red blood cell counts are associated with lupus)
  • Biochemistry to show the balance of electrolytes in the body (which can indicate abnormalities with the function of the kidneys and other internal organs)
  • Erythrocyte sedimentation rate (ESR) and/or C-reactive protein – elevated levels can indicate inflammation in the body
  • Tests to detect the presence of autoantibodies, which can indicate presence of an autoimmune condition
  • Coagulation studies that measure the clotting ability of the blood. Abnormalities can be associated with an increased risk of blood clots in some people with lupus.

Imaging studies such as X-rays and computerised tomography (CT) scans to reveal inflammation in the lungs and joints, magnetic resonance imaging (MRI) to test for inflammation in the brain and central nervous system, and echocardiograms and MRI to check for problems with the heart.

If lupus is suspected or confirmed, a referral to a specialist (usually a rheumatologist) is likely to 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. 

Treatment options include:

Medications

  • Non-steroidal anti-inflammatory drugs (NSAID's) such as ibuprofen and diclofenac, can help to reduce pain and inflammation
  • Corticosteroid medications are commonly used in the short-term treatment of moderate to severe cases of lupus and work by suppressing inflammation. Examples include prednisone, hydrocortisone, and dexamethasone
  • Antimalarial medications may be used to reduce your body’s autoimmune response. An example commonly used in the treatment of lupus is hydroxychloroquine
  • Immunosuppressive medications help to dampen down the abnormal response of the immune system and are used for treating people with more severe disease, such as when there is damage to internal organs. Examples of these medications include azathioprine and methotrexate
  • Biological medications may be used to treat severe cases of lupus that have not responded to other treatments. Rituximab is a synthetic (man-made) antibody that suppresses specific components of immune system function
  • Immune globulin may be given in severe flares of lupus.  It is a solution of antibodies made from human plasma, that is given intravenously.

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 regimen. When lupus is more problematic, more rest is required.

Avoiding sunlight

Because sunlight can trigger flares and skin rashes in some people with lupus, 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 (SPF) of 50+ should be worn when in the sun.
 
Give up smoking

Smokers with lupus should quit smoking. Smoking increases a person's risk of cardiovascular disease and can worsen the damage to heart and blood vessels caused by lupus.
 
Alternative therapies and diet

Reducing stress is vital for people with lupus and many people find that relaxation techniques such as meditation, yoga, and hypnotherapy can assist with stress reduction. 

Dietary factors are also important and a diet low in red meat and dairy products is thought to help reduce pain and inflammation. Some foods, such as alfalfa sprouts, and dietary supplements, such as Echinacea, should be avoided as they may cause lupus flares.

It is important to discuss any dietary changes or alternative therapies with your doctor or specialist before undertaking them to ensure 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:
Freephone: 0800 663 463
Email: info@arthritis.org.nz
Website: www.arthritis.org.nz 

References 

Arthritis New Zealand (2016). Lupus (Pamphlet PDF). Wellington: Arthritis Foundation of New Zealand. https://www.arthritis.org.nz/pdfs/brochures/Lupus.pdf
Bartels, C.M. (2020). Systemic lupus erythematosus (SLE) (Web Page). Medscape Drugs and Diseases. New York, NY: WebMD LLC. https://emedicine.medscape.com/article/332244-overview [Accessed: 02/09/20]
MedlinePlus (2020). Systemic lupus erythematosus (Web Page). Bethesda, MD: US National Library of Medicine (NIH). https://medlineplus.gov/ency/article/007113.htm https://medlineplus.gov/ency/article/000435.htm [Accessed: 02/09/20]
O’Toole, M.T. (Ed.). (2017). Systemic lupus erythematous (SLE). Mosby’s Dictionary of Medicine, Nursing & Health Professions (10th ed.). St Louis, MI: Elsevier.
 
Last reviewed: September 2020 

 
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