Historically, [lupus] has been responsible for a high numbers of fatalities. However, medical advances have succeeded in turning this situation around. Dying from lupus – at least in Western countries – is extremely unusual nowadays. Professor Graham Hughes
Professor Graham Hughes, Head of the London Lupus Centre, explains why he is optimistic that a cure for lupus will be discovered within the next 20 years…
In 1983, a team of scientists from St Thomas’ Hospital in London provided the first-ever description of antiphospholipid syndrome (APS). APS, which is also known as Hughes syndrome, is closely linked to lupus erythematosus: a group of autoimmune conditions with the capacity to disrupt any organ in the human body.
If left untreated, lupus can prove fatal, and because its variants manifest themselves in so many different ways, it has been historically difficult to diagnose and to treat. Indeed, in some regions of the developing world, lupus-related diseases continue to claim lives.
Even so, major strides have been made in the fight against this condition, and it is now rare for individuals living in Western countries to die from lupus. Although there is still no known cure, advances in the diagnosis and treatment of lupus have enabled medical professionals to significantly mitigate its impact.
This week marks the 30th anniversary of the discovery of Hughes syndrome. To honour this important achievement and to discuss the current landscape of related research, St Thomas’ will today host a meeting of interested specialists from around the world. Whilst significant progress has been made in the war against lupus, there is still work to be done.
To learn more about the ever-improving prospects of those with lupus-related diseases, I spoke to the pioneering rheumatologist after whom Hughes syndrome is named. In the following interview with ScienceOmega.com, Professor Graham Hughes, Head of the London Lupus Centre, discusses progress that has been made in the fight against lupus, and explains why he is optimistic that a cure for the condition will be discovered within the next two decades…
Could you begin by detailing how lupus affects sufferers?
Essentially, lupus is a condition that causes the immune system to become overactive. The immune system produces antibodies, and these have the potential to adversely impact organs and circulation. On the whole, lupus is a disease of young women; it affects nine times more females than it does males. As the condition causes huge numbers of antibodies to be produced, it has the capacity to harm pretty much every organ in the body.
I understand that there are several variants of this condition. Are there any symptoms that are common across all forms of lupus?
There are three main types of lupus: systemic lupus erythematosus (SLE), discoid lupus erythematosus (DLE), and Hughes syndrome – the variant that we first described 30 years ago this week.
All forms of lupus cause fatigue. The variants are characterised by non-specific features, which is why it can take some time for diagnoses to be made. The classical picture, if you like, is a woman of child-bearing age – perhaps 28 years old – who is simply feeling unwell. She might be experiencing aches, pains, or fatigue, and rashes are sometimes – but not always – present. The problem is that if left untreated, lupus can result in more serious consequences. It can cause damage to vital organs including the lungs, the kidneys, and even the brain.
As a result, lupus has garnered a fearsome reputation over the years. Historically, it has been responsible for a high numbers of fatalities. However, medical advances have succeeded in turning this situation around. Dying from lupus – at least in Western countries – is extremely unusual nowadays.
How much is understood about the mechanisms that cause the immune system to malfunction in this way?
We now understand a huge amount about this process. Firstly, there is a genetic tendency for lupus; we see the condition running in families. The earliest signs usually manifest themselves during the teenage years. It is at this stage that patients begin to develop B cells: lymphocyte blood cells that produce antibodies. One of these antibodies is known as anti-DNA.
Anti-DNA is an antibody specific to lupus, and it has two dangerous effects. Firstly, it prevents waste products from being cleared adequately which leads to an accumulation of toxic products. Secondly, the overproduction of antibodies often causes blood vessels to attach to sensitive areas such as kidney filters. ‘Sticky blood’ is actually the main characteristic associated with Hughes syndrome.
How have the prospects of lupus sufferers improved during the course of your career?
Prospects have improved dramatically. In the old days, there were two main causes of death. First and foremost was the failure to diagnose the condition. Sadly, figures for non-diagnosis of lupus remain relatively high in countries such as Guam and Indonesia.
People also died because of the infections and immunosuppression that resulted from the over-prescription of steroids. In fact, one of the biggest advances during my career has been the fine tuning of lupus treatment. In the past, patients were frequently administered high doses of steroids and immunosuppressants, and this had the effect of overcompensating for the original problem. Patients’ immune systems were flattened and they contracted infections. Nowadays, lupus is treated much more effectively, and as female patients reach menopause, the risk of dangerous lupus actually reduces. It is very unusual to see a new flare of lupus in a woman over the age of 50.
Do you think that we will find a cure for lupus?
Doctors tend to err on the side of caution when it comes to matters such as this, but I am very optimistic that a cure will be found. Just look at how much progress has been made. I would say that we already have about 95 per cent of a cure. However, there are still a few challenges that we must overcome in order to completely eradicate lupus.
When do you expect a cure to be discovered?
I am confident that a cure for lupus will be developed within the next 20 years. For the first time in my career, drugs are being created specifically for the treatment of lupus. Rituximab (RTX) and Belimumab, for example, are biological agents that only attack patients’ B cells. This has the effect of switching off antibody production. Belimumab is the first lupus-specific drug to gain the US Food and Drug Administration’s (FDA) seal of approval. We’re not on our own any more; the pharmaceutical companies are producing medicines tailored to this condition. I don’t think it will be too much longer, therefore, before a cure for lupus is discovered.
If you’d like to know more about the effects of lupus and the research that is taking place within this field, take a look at the London Lupus Centre’s website…
Oh Dear, If this is the 'Last Word on Science,' it's the last time I can take Omega seriously. 'Growing Credibility' ? Hardly. 'Certain Sections' [of the Scientific Community] 'beginning to question the longevity of death...' I suppose there may be Voodoo Sections in the community. I could go on, through the could's, untils, aims, hopefullys - but is there really any point?