19th March 2013

Behçet's disease, systemic vasculitis, auto-immune disease, Myalgic Encephalomyelitis and Chronic Fatigue Syndrome

By Stephen Ralph DCR(D) Retired.

 

For those of you who have been following my investigations regarding Behçet's disease, I have been following a trail that has a fundamental importance to the concept of "medically unexplained physical symptoms (MUPS)” and the possible uninvestigated and un-researched cause or causes of Chronic Fatigue Syndrome (CFS) and Myalgic Encephalomyelitis (ME).

Behçet's disease was recognised back in 1937 and was known as the "Silk Route" disease.

http://en.wikipedia.org/wiki/Behcet%27s_disease

Also referred to as systemic vasculitis, Behçet's disease (BD) is a self-perpetuating and lifelong auto-immune disease.

There are many different medical conditions placed under the heading of systemic vasculitis of which Behçet's disease is just one.

Check out this web site http://en.wikipedia.org/wiki/Systemic_vasculitis to see the other conditions that come under the heading of systemic vasculitis.

Behçet's disease can be triggered by a viral illness and it follows a recovery relapse course or can be continually disabling.

Systemic vasculitis happens when your immune system goes into overdrive and starts attacking your blood vessels anywhere in your body.

The inflammatory process can bring dysfunction to any bodily organ or system from your brain to your blood supply, your lungs, your heart, eyes, bowels, your bony joint spaces and your muscles.

All the above can be affected by an overactive inflammatory response to physical or even mental exertion causing a host of symptoms that are remarkably similar to those of Myalgic Encephalomyelitis or Chronic Fatigue Syndrome.

Here are some of those common symptoms....



• PROFOUND FATIGUE, BREATHLESSNESS AND MALAISE WITH PHYSICAL OR MENTAL
EXERTION

• INTERMITTENT FLU LIKE FEVER – HOT AND COLD FLUSH

• SORE THROAT, ENLARGED GLANDS IN NECK

• POST EXERTION FATIGUE - RECOVERY IS PROLONGED AND FRAGILE

• MULTI MUSCLE PAIN OR POLY-MYALGIA - LINKED TO PHYSICAL EXERTION

• MULTI JOINT PAIN OR POLY-ARTHROPATHY - LINKED TO PHYSICAL EXERTION

• COGNITIVE DYSFUNCTION – “FOGGY BRAIN”, POOR SHORT TERM MEMORY, PHYSICAL
CLUMSINESS, DIZZY SPELLS

• EMOTIONAL LABILITY, SHORT TEMPER, PERSONALITY CHANGES

• RECURRENT PROLONGED MIGRAINE LIKE HEADACHES

• HYPERSENSITIVITY TO LIGHT AND TO SOUND

• GASTRO-INTESTINAL DYSFUNCTION – IRRITABLE BOWEL SYNDROME

 

There are different "levels" of diagnosis for Behçet's disease.

http://www.behcets.org.uk/menus/main.asp?PN=medical-information-for-patients-how-diagnosed

The top most level of diagnosis includes patients who display visible "hallmark" signs of the disease including positive pathology on brain MRI scans, uveitis or inflammation in the eye, mouth ulcers, genital ulcers, skin lesions and a positive "pathergy" test skin reaction along with other visible signs and symptoms.

Research into Behçet's disease reflects the signs and symptoms relating to that "top tier" of diagnostic presentation.

As a consequence, this means that when any doctors be they GP's or general specialists go looking for diagnostic guidance, they read research papers that describe a "top tier" presentation for a disease that their patient may not be presenting at their GP surgery or their out-patient clinic.

Underneath that "elite" "top tier" are the rest of the Behçet's patient group and at the bottom of the list are patients who may have few or even no visible signs of Behçet's disease at an out-patient appointment. Those "bottom of the list" patients still have a catalogue of symptoms together with a significant disability because of those "invisible" symptoms.

One of those patient's was me.

Those patients may well have Behçet's disease but because the examining clinician is poorly educated to recognise the full spectrum of presentation of this disease, that patient may well be turned away or be told that they must have Chronic Fatigue Syndrome.

During my recent exchange of e.mail with Professor Sir Simon Wessely, he told me that he was not aware that the visible signs of Behçet's disease (that cause respective invisible symptoms) do not in fact need to be present for those "invisible" symptoms listed above to continue without a doctor being able to visually detect them.

As an example of this I directed Professor Wessely to this web page....

http://www.behcets.org.uk/menus/main.asp?PN=medical-information-for-patients-headache

This page describes the pathological abnormalities that can be observed in the brain on MRI scans of patients who suffer systemic vasculitis.

One of the symptoms that arises from those signs is a serious condition known as "secondary headache".

Secondary headache only occurs in a relative minority of Behçet's patients and the article goes on to describe "primary headache" syndrome that so far has not been clinically detectable.

On the other hand "primary headache syndrome" has been seen to be significantly disabling in those who suffer such primary headaches.

This same pattern can be seen in other bodily organs. The gastrointestinal tract can be affected by numerous painful ulcers. If those ulcers are not treated then they can perforate leading to an acute medical emergency situation.

However, a patient with Behçet's disease may not have any history of gastrointestinal ulcers yet they still suffer gastrointestinal dysfunction because the "sub-visible" disease activity attacking the gastrointestinal tract still causes gastrointestinal dysfunction that can be mistaken for irritable bowel syndrome.

Professor Sir Simon Wessely was not aware of these facts.

Going through the list of visible signs we come to Uveitis and hypersensitivity to light and to sound.

In some patients with Behçet's disease, patients can have obvious ongoing signs of eye inflammation or uveitis where the eyes of those patients look permanently bloodshot.

Some patients may suffer tiny blood clots because the inflammatory process affects the blood and creates blood clots that can cause all sorts of damage.

In relation to the eyes, tiny blood clots can cause partial or full blindness.

However, those people affected this way are a relatively small minority.

The rest of the Behçet's patient group may not show obvious signs of uveitis or eye disease but nevertheless, systemic vasculitis can still cause "invisible" symptoms that affect your eyesight in other ways such as hypersensitivity to light also called photophobia.

From my exchange of e.mail's with Professor Sir Simon Wessely, he did not seem to know about any of this at all.

Professor Wessely said to me that he was "not a Behçet's specialist" and that he would need to phone a colleague who was a Behçet's specialist to find out more.

Professor Wessely also said that he was going to task a medical student to do some research for him into systemic vasculitis and Behçet's disease.

Professor Wessely was not aware that patients at the opposite end of the diagnostic spectrum for Behçet's resemble people diagnosed with Myalgic Encephalomyelitis or alternatively Chronic Fatigue Syndrome.

At first Professor Wessely tried to assert that he thought I was talking about something called "diagnostic overshadowing" whereby a primary diagnosis of Chronic Fatigue Syndrome could "overshadow" the symptoms of another pathology such as heart disease or cancer.

I broke it to Professor Wessely that what I was actually trying to establish was why he or his colleagues, over a period of more than thirty years had never rigorously looked at, considered, investigated or excluded the pathological processes of systemic vasculitis as a possible explanation for the perpetuation of the invisible symptom set that is common to both systemic vasculitis and Myalgic Encephalomyelitis and Chronic Fatigue Syndrome.

If a group of doctors - in this case a group of "liaison psychiatrists" are going to define an artificial construct of "medically unexplained physical symptoms" and then create a group of patients under that artificial banner including patients labelled as having Chronic Fatigue Syndrome or Fibromyalgia Syndrome et al then that group of doctors are duty bound by their professional standards to ensure that they have first rigorously gone through, considered, investigated and excluded all the existing possible alternative explanations for what they decide to term "medically unexplained
physical symptoms."

I have now learned that Professor Sir Simon Wessely has never done this and as far as I can tell, neither have any of his like minded colleagues such as Professor Michael Sharpe, Professor Peter White OBE, Professor Trudy Chalder or others like Dr Vincent Deary or Dr Esther Crawley.

If they have been involved in such rigorous and extensive investigations then we have never been told about such an extensive exclusion process.

Could such a profound failure to investigate all the pre-existing possible alternative explanations for the underlying cause or causes of Myalgic Encephalomyelitis or Chronic Fatigue Syndrome be considered to be collective medical negligence? Could their collective behaviour surrounding "medically unexplained physical symptoms" be quietly constructive and if so what motivated this group of psychiatrists to ignore clinical explanations and instead smother a group of sick and disabled patients with psycho-social explanations?

The group of patients who represent the vast majority of only two handfuls of clinical research studies into Behçet's disease, all meet the "top tier" diagnosis for Behçet's disease where those patients have all the obvious "hallmark" visible signs of the disease.

By stark contrast, the patients who meet the diagnostic criteria yet who sit at the other end of that diagnostic spectrum are not represented by research at all because it seems that nobody has bothered to look into why the other end of the Behçet's population still suffer considerably whilst not showing the typical "hallmark" visible signs..

Likewise, those who suffer severe Myalgic Encephalomyelitis or severe Chronic Fatigue Syndrome have never been involved in any substantial contemporary MRC funded or Government approved research.

Nobody has ever looked at the incredible overlay of invisible or hidden symptoms that are shared by people suffering from systemic vasculitis and Myalgic Encephalomyelitis (ME) or Chronic Fatigue Syndrome (CFS).

I have told Professor Wessely that it is possible to have systemic vasculitis without the disease itself eliciting many or indeed any examinable or testable visible signs.

Behçet’s disease is often diagnosed on the basis of a regular pattern of physical symptoms (listed above) that last for more than six months together with some visible signs such as 4 or more mouth ulcers in any 12 month period and skin lesions that look like acne.

Myalgic Encephalomyelitis and Chronic Fatigue Syndrome is diagnosed in exactly the same way and a patient may well suffer from mouth ulcers that a doctor will never ask a patient about.

Patients may also suffer from acne that is wrongly diagnosed as (adult) late onset acne.

Further to this, it is known that Behçet's disease can develop during the late 20's and early 30's of an adult's life. Therefore a patient could have been fit and healthy and be getting on in their life and their career when all of a sudden and without any apparent cause, that patient will suddenly "crash" and become seriously ill and disabled with no apparent reason.

Because there are no reliable tests to diagnose Behçet's disease, the usually array of standard tests will frequently come back negative leaving the GP or the general rheumatologist at a loss to see what is actually wrong with that patient.

This is why a patient is dangerously vulnerable to being subjected to a medical misdiagnosis and one of those patients was me.

With thanks to Professor Sir Simon Wessely and his like minded colleagues, GP's and general specialists all around the UK were given the option of giving patients a diagnosis of Chronic Fatigue Syndrome or Post Viral Chronic Fatigue Syndrome if a virus had occurred at around the time that the patient "crashed" and stopped being able to function.

I put it to Professor Sir Simon Wessely that I could make a valid claim that Myalgic Encephalomyelitis and Chronic Fatigue Syndrome could be a new variant of systemic vasculitis; a variant of this disease that does not elicit visible signs.

Professor Sir Simon Wessely did not reply to that suggestion.

At present due to the ongoing failure of clinical research, nobody responding to my claim could say that I was wrong in my beliefs.

However, Professor Wessely did tell me that his view was that it was up to other parts of the medical profession to undertake such investigative research to find any possible link between auto-immune systemic vasculitis and the possible underlying cause or causes of Chronic Fatigue Syndrome and Myalgic Encephalomyelitis.

I found this statement quite astonishing because putting it bluntly, if the rest of the medical profession working in immunology or rheumatology together with the Government, the Medical Research Council and the National Institute for Health and Clinical Excellence decides not to carry out any research into auto-immune disease as a potential underlying cause of CFS and ME; psychiatrists such as Professor Sir Simon Wessely, Professor Michael Sharpe, Professor Peter White, Professor Trudy Chalder, Dr Vincent Deary, Dr Anthony Cleare or Dr Esther Crawley can all carry on claiming in lectures and peer reviewed research papers that the symptoms of CFS and ME amount to a psychosocial disorder of "medically unexplained physical symptoms."

It appears to me that the involvement of "liaison psychiatry" and all those psychiatrists cited in this article have opportunistically grasped hold of "medically unexplained physical symptoms" because biomedical clinical research has simply failed to get around to medically explaining a set of symptoms that are already explainable when applied to other diseases such as Behçet's disease. It is a fact that clinical research has failed in it's duty in this respect for more than 30 years.

Subsequent to my exchange of e.mail with Professor Wessely, I also sent out individual e.mail's to all the other protagonists involved here in the UK including those psychiatrist already cited above in this essay.

I asked them to comment on the issue of auto-immune disease, systemic vasculitis and Behçet's disease in relation to Myalgic Encephalomyelitis and Chronic Fatigue Syndrome.

So far I have not had any responses to my questions.

The list of symptoms given above cannot be simultaneously "medically unexplained physical symptoms" in one breath and then "fully medically explainable physical symptoms" in the next breath.

Specifically, and in relation to CFS, ME and Behçet's disease are the obscure yet common symptoms of hypersensitivity to light (photophobia) and hypersensitivity to sound (phonophobia) in relation to all the other shared correlating symptoms. This is a fascinating aspect that at present nobody has explored or explained.

Having learned a great deal about how remote and detached Professor Wessely is with regards to caring about looking at potential underlying biomedical causes of CFS and ME; allowing him to perpetuate the concept of "medically unexplained physical symptoms" - I now strongly feel that Professor Wessely and his like minded colleagues cited above could all have professional conflicts of interests in wishing any such research to take place.

Should future research into CFS, ME and other MUPS disorders find any association with systemic vasculitis or auto-immune disease and the underlying self perpetuating causes of Myalgic Encephalomyelitis and Chronic Fatigue Syndrome then such research would find that the symptoms of ME and CFS were not in fact medically unexplained physical symptoms at all.

Such research could terminate the careers of those who have spent up to 30 years promoting the concept of "medically unexplained physical symptoms", somatoform disorders and most recently Somatic Symptom Disorder (SSD) http://dxrevisionwatch.com/tag/suzy-chapman/.

Such investigative research could have taken place 30 years ago or 20 years ago or 10 years ago or even 5 or less years ago.

The fact is that here in the UK, the vast majority of research funding has been diverted into a "dead-end" channel exploring only two issues involving the benign treatments of Cognitive Behavioural Therapy and Graded Exercise Therapy in relation to the psychosocial beliefs of Chronic Fatigue Syndrome.

As we have seen from the PACE Trial findings, those therapies have failed in the majority of cases to provide permanent functional rehabilitation for any of those who participated.

Those research monies could have been directed at research trying to establish underlying causes of a set of extremely disabling and distressing self-perpetuating symptoms (shared by people suffering other pre-existing diseases such as Behçet's disease) that have and still are confining victims to their beds, pushing those victims over the edge where they cannot cope anymore and commit suicide or spend years not being able to function or work or live as healthy people are able to live.

As a retired diagnostic radiographer who had it drummed into me that the accuracy of the diagnosis was the all important issue relevant to subsequent treatment and care, it is my view that the creation of "medically unexplained physical symptoms" was nothing more than an enabler of the "dumbing down" of clinical diagnosis itself not just in this country but around the world.

The new and recent invention of Somatic Symptom Disorder (SSD) has effectively dumbed down even further the diagnostic process offered to any patient who may present with a complex biomedical disease that will not reveal itself through a set of routine tests or scans.

When a patient goes to their GP with a set of "invisible" symptoms that show up negative on these simple tests, the diagnosis of Chronic Fatigue Syndrome was created to feed those patients into a diagnostic cul-de-sac when a doctor ran out of any other ideas or had only a limited knowledge of the wide presentation of rare pathological diseases.

Additionally, such a diagnosis is relatively inexpensive compared to an alternative diagnosis of Behçet's disease for example where expensive drugs are needed to suppress and down regulate a perpetually overactive immune system.

In my own case, I just happened to change my GP and my new GP just happened to have a personal friend who suffered from Behçet's disease.

That GP saw an alternative explanation for my set of "invisible" symptoms along with a few photographs I supplied him of my knees.

Prior to this I had seen nearly a dozen doctors and none of them be they GP's or general specialists had a clue about Behçet's disease or systemic vasculitis.

I was never asked about the visible signs of Behçet's disease even though I later realised that I had experienced symptoms that were specific to Behçet's disease such as periodic epididymitis that was observed by a GP who associated it with nothing else even though I was forced to take sick leave from work with symptoms that were extremely painful and exhausting.

I was medically misdiagnosed by one GP and two general rheumatologists but it was the knowledge of a subsequent GP eight years down the line that changed my life.

This is where Dr Clare Gerada becomes involved with the culture of GP's and the diagnosis of medical conditions that come under the artificial umbrella of "medically unexplained physical symptoms."

Dr Clare Gerada is the head of the Royal College of General Practitioners.

Dr Gerada is also the wife of the chief proponent of the concept of "medically unexplained physical symptoms" and Chronic Fatigue Syndrome - Professor Sir Simon Wessely.

Too many GP's know so little of the presentation of rare pathologies yet over the last 20 years GP's have been "guided" and saturated by information supplied by research into "medically unexplained physical symptoms" (MUPS) and Chronic Fatigue Syndrome.

Since 2010, Dr Clare Gerada MBE has been the Chairwoman of the Royal College of General Practitioners. Dr Gerada is also the wife of Professor Sir Simon Wessely.

http://en.wikipedia.org/wiki/Clare_Gerada

It has now become incredibly easy for a medical misdiagnosis to be made by any General Practitioner because the GP may not know about the striking set of symptoms relating to something like systemic vasculitis on one hand but on the other hand they will know all about the research and the diagnosis of Chronic Fatigue Syndrome published by Professor Sir Simon Wessely and his like minded colleagues who are all members of the "MUPS" project started more than 30 years ago.

The confidence a GP may have in the validity of CFS or ME as a psychosocial disorder is in my view compounded by the fact that Dr Clare Gerada - wife of Professor Sir Simon Wessely - is the Chair of the Royal College of GP's and previously trained in psychiatry and worked at the Maudsley Hospital..

I am just grateful that I now have a drug called Pizotifen that helps me a great deal with my primary headache syndrome.

Those who have severe Myalgic Encephalomyelitis or severe Chronic Fatigue Syndrome and suffer similar headaches, photophobia and phonophobia - are probably not being prescribed Pizotifen because the "psychosocial" concept of their diagnosis means that their GP or their general specialist may not fully appreciate the severity and frequency of those headaches or why they are caused. The causes of those remarkably similar severe Behçet's headaches in relation to CFS and ME are perpetually being described as "medically unexplained."

With regards to Professor Sir Simon Wessely and his like minded colleagues including Professor Michael Sharpe Professor Peter White, Professor Trudy Chalder, Dr Vincent Deary, Dr Esther Crawley et al; if none of them have periodically, systematically and thoroughly excluded Behçet's disease or systemic vasculitis as being a possible underlying cause for CFS, ME or other MUPS related disorders then how many other medical conditions (that share a significant number of common "invisible" symptoms) have this group of psychiatrists failed to investigate, consider and then exclude over a period of more than 30 years and counting.......?

After all, according to Professor Sir Simon Wessely that research is not his responsibility and it is not the responsibility of his like minded colleagues promoting MUPS and SSD – it is as I have now learned - the responsibility of others.

Where does a diagnosis of CFS or ME end and where does a diagnosis of Behçet's disease begin? At present nobody can say because there has been no clinical research.

How many other patients are living with a medical misdiagnosis around the world due to a failure of clinical research and the opportunism of "liaison psychiatry"?

How angry must that make those patients involved - feel?

Yours sincerely,

Stephen Ralph DCR(D) Retired.

www.meactionuk.org.uk

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To: MEActionUK@yahoogroups.com
Sent: Tuesday, December 18, 2012 11:35 AM

How can there POSSIBLY be a set of symptoms which are not ultimately medically explainable? Every response in the body is biologically explainable if the science is done, in the same way all the physical world is explainable. Anything in the physical world not explainable by science we call supernatural or paranormal or miraculous.

Even if the ultimate cause of those symptoms is a way of thinking (which I believe is not proven possible) the biological pathways are demonstrable, or will be as more and more of the body's workings are teased out by proper research.

So "medically unexplained" is a statement about the medical world NOT a statement about what is physically wrong with the patient. It is a statement of the inadequacies of research into disease not an answer to what the disease is and says NOTHING about the patient.

To say "We have not deciphered hieroglyphics yet" does not mean the Ancient Egyptians wrote nonsense on their walls, it reflects an ignorance at a point in time. When the answer was found all the writing was understood. The walls and writings stayed the same, only the state of knowledge changed.

Also, even if the CAUSE of symptoms is a way of thinking, there is no evidence whatsoever that a change of thinking will cure the disease.

Smoking may cause your lung cancer, but giving up smoking will not cure it. The lack of logic shown by these people is horrendous.

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