The disease with many faces: Systematic Lupus Erythematosus (SLE)

Systematic Lupus Erythematosus (SLE)

Lupus is a chronic inflammatory disease that occurs when the body’s immune system attacks tissues and organs. The form of lupus ‘Systematic Lupus Erythematosus’ (SLE), makes up 70% of cases.

The World Health Organisation (WHO) have previously stated that cases of Lupus have almost tripled over the past four decades, whilst the 50% ten-year survival rate has increased to 80-90%. Although this disease has no cure, with treatment the condition can improve especially when treated early.

Symptoms: the disease with many faces

Over the years, SLE has been referred to as ‘the disease with many faces’ as a result of the symptoms being commonly found in other illnesses, including infections and malignancies.

There are three main symptoms of SLE:

  • Joint pains/stiffness
  • Extreme tiredness
  • Skin rashes that are often over the nose and cheeks

However, there are a variety of other symptoms, including:

  • Weight loss;
  • Swollen glands;
  • Sensitivity to light; and
  • Poor circulation in fingers and toes.

Visible symptoms, such as rashes and swelling, reflect widespread inflammation of blood vessels occurring throughout the body.

Furthermore, there are also three types of severity when it comes to SLE: mild, moderate, and severe. Mild SLE typically includes tiredness and joint and skin problems, whereas moderate SLE tends to cause inflammation of other parts of the skin and body, including the lungs, heart and kidneys. However, severe lupus involves inflammation causing serve damage to the heart, lungs, brain and kidneys, which can potentially be life threating.
Moreover, previous studies have found that inflammatory musculosketal features, such as joint pains, are present as one of the first symptoms in 50% of patients and 95% of all SLE patients will be affected by this at some point.

As well as physical symptoms, SLE has also been related to mental illness, with one study finding that 26% of children and 44% of young adults with SLE have symptoms of depression. There is also said to be a frequent occurrence of cognitive disfunction amongst those with this form of Lupus, ranging in severity from mild impairment to severe dementia; this includes impairments in attention, recall, concentration, information processing speed, spatial processing and word finding. However, in terms of SLE and cognitive disfunction, currently the screening, diagnosis, and monitoring of these symptoms are under developed.

Causes and diagnosis

Unfortunately, SLE is prone to delayed diagnosis and is said to be one of the ten most mis-diagnosed diseases by doctors. Research proposes that there are 50,000 people in the UK living with lupus and, on average, there is a seven years’ time difference between SLE symptoms onset to diagnosis. There are variety of reasons for this, including the fact that many conditions mimic SLE symptoms, such as tissue and hemiplegic diseases, and also the fact that symptoms can be vague and develop gradually over time. There also tends to be phases of worsening symptoms that alternate with periods of mild symptoms. However, typically, if blood tests show a high level of antibodies combined with typical symptoms, then a diagnosis of SLE is likely.

The causes of SLE are not yet fully understood. However there are several possible triggers; these include viral infections, strong medication, sunlight, puberty, childbirth and menopause. In accordance to this, because there have been significant findings that 90% of SLE is found in women aged between 15 and 34, researchers believe that there may be a possible link between lupus and oestrogen, resulting in the suggestion that there may be a possibility that hormones play a role.

Indeed, SLE is nine times more likely to occur in women than men and is three to four times more likely to be found in black and Asian women. As SLE is an autoimmune disease, meaning that the immune system attacks the body because it confuses it for something foreign, there is not a specific age group who suffer with SLE. However, it has been found to be unusual in those under five years old and those older than 50. Moreover, although there is not thought to be a specific ‘lupus’ gene, it is believed that SLE can run in families, and this this would suggest that genes do play an important role in the development of the disease.

Treatment

After diagnosis, regular checks and tests are advised. This could include regular blood tests for anaemia and urine tests to check for kidney problems which could be caused by SLE. There are many treatments which have been shown to be effective in lupus-prone mice, however treatment in humans is not as simple. Here, there are a variety of options. These includes anti-inflammatory medications, such as ibuprofen, hydroxychloroquine for fatigue, skin and joint problems and steroid tablets, injections and creams for kidney inflammation and rashes. Interestingly, antimalarials such as hydroxychloroquine, which is a systematic inflammation direct treatment, is used for mild forms of SLE, but while this has been shown to be effective in cases of as mild severity, this form of treatment has been deemed ineffective in preventing the occurrence of more severe SLE manifestations.

More recently however, two new drugs have been introduced; rituximab and belimumab. However, limitations of these medications include that as SLE patients are prone to infections, this risk can be heightened because of the combination between their abnormal immune system and drugs that involve immune supressing properties to control SLE activities.

In addition to these treatments, there are also certain ways SLE patients can manage their symptoms. That is, SLE experts have said that the most important thing to do when diagnosed with lupus is to stop smoking and it is also advised that those with SLE do not sit in direct sunlight or spend a lot of time in rooms with fluorescent lights.

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