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Many of the chronic conditions we’ve discussed here on the blog, whether they’ve been high blood pressure, diabetes or glaucoma, have shared a common characteristic: early symptoms of these potentially very serious conditions can often be easy for patients to miss.

However, doctors and specialists will often be able to recognise and diagnose the above to a degree of certainty, after carrying out one or two tests.

This isn’t always the case with the subject of this week’s post: systemic lupus erythematosus (SLE), more commonly referred to as lupus. As we’ll discuss, the condition can be complicated, difficult to identify without extensive testing, and one which can be prone to misdiagnosis from doctors.

Lupus Awareness Month is a campaign which takes place every October, and is run by Lupus UK. It aims to increase understanding of the condition, not just among the general public, but among healthcare professionals too.

With just over four weeks to go until the campaign gets underway, this week we talk to Paul Howard, Social Media and Projects Manager from Lupus UK, to discuss lupus in more detail: specifically who it affects, what diagnosis and treatment involves, and what research is being undertaken to further the development of new treatment methods.

How common is lupus, and who does it affect?

Lupus isn’t very common. The NHS estimates that around 15,000 people in England and Wales have SLE and women, as Paul explains, account for the majority of these cases:

‘Lupus can affect anybody, but it is much more common in women with approximately 90% of cases being female. Women are most commonly diagnosed during childbearing age (15-45) but some studies have suggested that up to 20% of cases develop in childhood.

Persons from certain ethnic backgrounds are also thought to be more likely to develop the condition, Paul goes on to say. Lupus is particularly prevalent among persons of African descent; including too those who have been born in a non-African country such as the UK or US, and have continue to live there for all their lives.

One analysis undertaken in Birmingham in the 1990s reported that, among those surveyed, lupus affected:

  • one out of 500 adult women of Afro-Caribbean descent
  • one out of 1,000 adult women of Indian or Pakistani descent
  • one in 2,500 of those of white European descent.

Furthermore, the condition is thought to affect one in 250 Jamaican women, and persons of Chinese and Polynesian ethnicity are also thought to be at increased risk.

Symptoms and seeing a doctor

Lupus is what’s known as an autoimmune condition. In this type of illness, the immune system, which is responsible for fighting off harmful pathogens (viruses and bacteria), essentially malfunctions and targets healthy cells or tissue in the body. This causes inflammation and sometimes lasting damage to the affected organ or tissue.

The symptoms of lupus can be wide-ranging, and vary from mild to severe. These may also differ significantly from person to person.

Some of the symptoms most commonly associated with lupus are:

  • extreme fatigue
  • rashes from exposure to UV light
  • butterfly rash over the cheeks and bridge of nose
  • and muscle and joint pain

But Paul explains that symptoms of the condition may also include:

  • oral or nasal ulcers
  • hair loss
  • flu-like symptoms
  • rashes from sunlight or UV light
  • kidney problems
  • heart and lung involvement
  • increased risk of miscarriage
  • depression
  • and headaches.

If somebody is experiencing multiple symptoms over a prolonged period of time and they cannot be satisfactorily explained (such as by a virus) then they may want to ask their GP to refer them to a rheumatologist to investigate the cause and perhaps test for lupus.’

‘Unfortunately there is often a significant delay in the diagnosis of lupus.’ Paul goes on to say. ‘In the UK it currently takes an average of approximately 7.5 years from the onset of the first symptoms to get a diagnosis. Better awareness of the condition by the public and medical professionals will hopefully reduce this delay significantly.

How serious is it?

Not everyone who has lupus will develop serious symptoms. But it can lead to severe health problems in some cases, as Paul illustrates:

Lupus is a very varied condition which affects each patient differently.

Those who are diagnosed usually receive ongoing treatment to lessen the impact of the many symptoms and reduce the risk of long term damage (though there may be side-effects). In severe cases (and sometimes when left untreated), lupus can potentially damage the kidneys, heart, lungs or brain and unfortunately in rare cases it can be fatal.’

The side effects of medicines used in treating lupus that Paul refers to, can themselves on occasion lead to serious health issues, which can make safe and effective management of the condition even more troublesome:

Some treatments used in lupus can unfortunately also have long-term health effects; high doses of steroids are a frequent culprit and can cause problems such as osteoporosis, obesity, cataracts, high blood pressure and diabetes.’

It’s therefore crucial for patients and doctors to remain in regular contact so that they can effectively monitor the condition and manage treatments as appropriate.

How often is lupus misdiagnosed?

As you might expect, because there are so many potential symptoms which lupus can cause, and no uniform consistency in which they may occur, it’s not uncommon for doctors to mistake lupus for other illnesses:

Due to the wide range of symptoms and their often fluctuating nature, lupus is frequently misdiagnosed.’ Paul tells us.

From a survey of LUPUS UK members, we learned that 45 percent are initially misdiagnosed with another condition first. The survey of over 3,000 people with lupus revealed over 30 different conditions that lupus had been mistaken for.’

Some conditions that may be commonly mistaken for lupus include:

  • chronic fatigue syndrome (CFS or ME)
  • depression
  • glandular fever
  • rheumatoid arthritis
  • rosacea
  • fibromyalgia
  • scleroderma
  • vasculitis
  • and multiple sclerosis.’

For a patient, going back to the doctor to raise concerns about what they feel could be a potential misdiagnosis can sometimes seem tricky. Obviously, GPs spend years being medically trained to identify and treat a wide range of health issues; and many patients may feel less inclined to question their judgment as a consequence.

But this shouldn’t stop patients voicing their concerns, as Paul explains:

‘If somebody suspects that they have been misdiagnosed and may have lupus, it can be helpful to discuss any symptoms which they feel don’t fit with current diagnosis with their doctor and ask to be tested for lupus.’

‘If it was a GP who made the initial diagnosis, it is often beneficial to ask for a referral to a rheumatologist who will often have more experience in diagnosing and treating rarer autoimmune conditions like lupus.’

Triggers and flare-ups

Little is known about what causes lupus, as is the case with so many other autoimmune conditions. More research is needed, but some theories suggest that the root cause may be down to genetics (lupus is thought to be more likely in the siblings and close relatives of an affected person) or certain environmental factors (such as hormonal fluctuations or exposure to UV).

As with other autoimmune illnesses, lupus may go through periods of flare-up and remission, with symptoms activated by certain triggers. These may again vary from patient to patient.

A common trigger for lupus appears to be hormonal changes.’ Paul tells us. ‘Most people with lupus are females who are diagnosed between the ages of 15-45, with many people being diagnosed during puberty or after childbirth.

It is also believed that certain viral infections, exposure to UV light and trauma also trigger lupus.’ Paul goes on to say. ‘Many people with lupus notice an increase (or ‘flare’) of their symptoms if they are exposed to sunlight or when they catch viruses such as the common cold or flu. In some cases people may experience flares with no discernible trigger.’

How is it diagnosed?

As Paul explains, testing for lupus isn’t as simple as it may be for other chronic conditions, where a diagnosis may be arrived at after using a single blood analysis or carrying out a scan:

Lupus doesn’t have a single blood test which will confirm a diagnosis, so instead diagnosis is made based on a range of blood tests, an examination of the patient and a discussion of family medical history (to ascertain if there is a history of autoimmune conditions).’

‘Many of the blood tests used in the diagnosis for lupus are looking for certain ‘autoantibodies’. These are antibodies produced by the body’s immune system to attack the body’s own cells instead of invading cells (such as viruses and bacteria).’

‘Some of these tests include:

  • antinuclear antibody (ANA) test,
  • anti-dsDNA antibody test,
  • ENA (extractable nuclear antigens) test
  • and antiphospholipid antibody test.

Paul goes onto say that these tests will usually be backed up with more common analyses, including a full blood count, as they are in other autoimmune conditions:

In addition to these tests, the doctor will usually request a full blood count and biochemistry. The blood count in lupus can show low white cells, low red cells and low platelet counts. Biochemical tests are important, especially the creatinine and urea which are raised if there has been evidence of kidney disease.’

The ESR and C-reactive protein (CRP) tests carried out from a blood sample, Paul tells us, are used to give an idea of how active the condition is.

How is it treated?

Currently, there is no way to cure lupus, and few medicines specifically designed to treat this condition alone. As Paul tells us, lupus is managed in a similar way to other autoimmune conditions; through one or more treatments aimed at reducing the harmful effects of autoimmune response on the body:

Common treatments for lupus include non-steroidal anti-inflammatory drugs (NSAIDs such as ibuprofen), anti-malarial treatments and steroids. In moderate and severe cases of lupus, immunosuppressant treatments may be used which can include low doses of chemotherapy treatments such as methotrexate.’

Many treatments prescribed for lupus are not actually licensed for lupus and were originally designed for other conditions, such as rheumatoid arthritis,’ Paul goes onto explain, ‘but these have proven to also benefit some people with lupus. Only one treatment specifically for lupus has become available in over 50 years; Benlysta (Belimumab) which is only now being accepted for limited use in the NHS in England and Wales after waiting five years for a decision from NICE.’

As some treatments used in lupus can often pose health risks of their own, patients taking them will often need to take supplements to offset the potential adverse effects they can cause:

Most people with lupus will also be required to take a range of other non-lupus treatments to help combat certain symptoms or side-effects from treatments. A common example of this is calcium and vitamin D (to try and limit the depletion of bones caused by steroids).’

Lifestyle changes

Managing lupus doesn’t stop at just medicine either. As with other conditions, maintaining a healthy outlook in everyday life plays a vital role in reducing the risk of flares and limiting symptoms:

Lifestyle adjustments are also very important in the management of lupus.’ Paul tells us. ‘Protecting oneself from exposure to the sun by using factor 50+ sunscreen and covering up is very important, as is eating a healthy, balanced diet and participating in regular physical activity.

It’s also crucial for those living with lupus to ensure they get enough rest when they need it, and not push themselves too hard:

Pacing is an extremely important practice for the many people with lupus who struggle with extreme fatigue.

Are there any new treatment methods on the horizon?

Lupus is by no means a new condition. It has apparently been observed by medical professionals for hundreds of years; however our understanding of lupus and effective treatment methods has only begun to develop more recently, in the past 50-60 years.

Extensive research into the condition, Paul explains, is ongoing:

A lot of research is currently being undertaken at multiple levels. Lupus is a very complicated condition, and our understanding of the causes and triggers needs to be improved in order to develop more innovative treatments and preventative measures, so a lot of research examining the genetics and immune cells of people with lupus is being carried out.’

And as you might expect, broadening the range of viable treatments for SLE, Paul tells us, is the desired outcome of this:

A wide number of new treatment options are in various stages of development, each targeting a different pathway in lupus. Also, a number of treatments which are currently licensed for other conditions are also receiving clinical trials to examine whether they may be beneficial in some cases of lupus.

One of the largest upcoming research studies in the UK is a project called MASTERPLANS which is a massive £4.1 million consortium study aiming to ‘stratify’ care for patients with lupus.’

Through the investigation of lupus ‘fingerprints’ (in that SLE affects individuals in differing ways and to varying degrees, so every patient essentially possesses their own ‘lupus fingerprint’), this project hopes to make the process by which treatments are chosen for individual cases more effective:

The aim of the study is to examine a range of variables which can influence the success or failure of treatment and identify key factors that can predict more accurately the correct treatment to offer to individual patients. You can read more about this on our website.’

Over on Lupus UK you’ll also find more information on the condition, a wide array of expert advice, and more on Lupus Awareness Month and how you can take part.