"Joint hypermobility: emerging disease or illness behaviour?"

[Kyla]

http://www.clinmed.rcpjournal.org/content/13/Suppl_6/s50.full

I had no idea that JHS and EDS were sometimes considered different diseases, or that there was so much controversy surrounding them. A very interesting read.

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Joint hypermobility syndrome is a common clinical entity which is much misunderstood, overlooked, misdiagnosed and mistreated. It was first described in the 1960s as a purely musculoskeletal condition due to joint laxity and hypermobility occurring in otherwise healthy individuals. Some four decades later it is now perceived to be a multi-systemic heritable disorder of connective tissue with manifestations occurring far beyond the confines of the locomotor system and with ramifications potentially affecting most, if not all, of the bodily systems in one way or another. Most authorities in the field find it clinically indistinguishable from the Ehlers-Danlos syndrome – hypermobility type (formerly, EDS type III). In >50% of patients the diagnosis is delayed for ≥10 years. Failure to diagnose and treat the condition correctly results in needless pain and suffering and in many patients to a progressive decline in their quality of life and in some to a loss of independence.

There is a common tendency in medicine when faced with medically unexplained symptoms to assume that they are psychogenic in origin. This is a high-risk approach that can have disastrous consequences. The joint hypermobility syndrome (JHS) story is a good illustration of this...

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...read the rest here:
http://www.clinmed.rcpjournal.org/content/13/Suppl_6/s50.full

 

[Sidereal]

With the passage of time and the advent of new knowledge, it has become clear to clinicians seeing many of these patients that, to all intents and purposes, the two diseases are indistinguishable from one another.3 The tragedy is that it took four decades to achieve this consensus. Sadly, even today, there are sceptics who do not yet accept this hypothesis and fervently advocate the separation of the two conditions. For them (principally, but not exclusively, rheumatologists), lax-jointed patients with HMS are ‘otherwise healthy subjects’ whose extra-articular symptoms are taken to represent illness behaviour, rather than bona fide symptoms of systemic complications of EDS III.

 

[Sidereal]

The natural history of JHS can be seen to comprise three principal phases. The first phase results from connective tissue laxity and fragility and comprises multiple soft tissue injuries, joint and spinal instability and dislocation, and complications of weakness in supporting structures (pelvic floor, herniae and varicose veins). Phase 2 is the superimposition of non-articular complications: pain amplification, kinesiophobia deconditioning (widespread chronic pain often described as ‘fibromyalgia) and fatigue (which is often prominent and may be misdiagnosed as CFS/ME). It is in this phase that orthostatic intolerance, PoTS and other dysautonomic features as well as GI symptoms make their presence felt. The final phase is the emergence of psychosocial sequelae such as anxiety or depression, obesity (often associated with comfort eating), work incapacity, isolation and despair. At this stage, there is often a downward spiral of loss of mobility, self-efficacy and self-esteem as the quality of life diminishes. The reasons for this decline are complex and multifactorial. At this stage, the patients are severely disabled in spite of the fact that their musculoskeletal system is usually grossly intact. It would appear that the combination of overriding chronic pain (which is largely unresponsive to analgesics) and systemic components (PoTS and GI problems) is the determining factor in this decline.

 

[SilverbladeTE]

you know....
when an astronomer spots a peculiarly bright, incredibly massive objects at extreme range...
they may at first think fault with their detection equipment, so check it
when it checks, they get others to check
when they also see same thing, they don't all say "IT'S IN THEIR HEADS!"
they try and find out what it is!
years later, it's called a "Quasar"
they still don't know what it is, but they agree it's a genuine phenomena
years later still, they believe such things are caused by enormous black holes sucking in the core of entire galaxies.
yes there ARE fights and issues over such, hey, Science is a *Human Cultural Endeavour* it is NOT the Method itself or remote cold perfect methodology.
But in most Science they aren't such arrogant jerks or cause other people's abuse and deaths
So why is medicine infested by so many DICKHEADS?!

 

[Jonathan Edwards]

http://www.clinmed.rcpjournal.org/content/13/Suppl_6/s50.full

I had no idea that JHS and EDS were sometimes considered different diseases, or that there was so much controversy surrounding them. A very interesting read.

...read the rest here:
http://www.clinmed.rcpjournal.org/content/13/Suppl_6/s50.full

What I think may be worth noting is that there is essentially no scientific basis in terms of referenced studies to what my dear friend Rodney is saying here. I admire his enthusiasm but where are the data? About the only piece of data you will find in the references in terms of documenting features of what was then 'benign hypermobility syndrome' is number 5, which you will note has my name on it! (And I was never very happy about the result we published.)

In those days we did a clinic for people with pain in hypermobile joints. There was no talk of pain elsewhere or widespread pain or POTS or anything. But once people got to know that there was a clinic for people with hypermobility and pain of course everyone with pain who happened to be hypermobile got sent in. So the clinic filled up with people with all sorts of pain and then when the word got about that there was a link between hypermobility and 'fibromyalgia' everyone with fibromyalgia and hypermobility was sent to the clinic from far and wide so Rodney found himself surrounded by people with the two problems and started writing articles about how often they occurred together. As far as I know no research was done to confirm this.

As I see it the bit about 'all in the mind' is a red herring here. All that we are interested in is whether widespread pain is causally related to hypermobility or whether, if they do occur together, it is an irrelevance. There are people with severe hypermobility who have dislocations, but I am unclear what that has to do with pain elsewhere. Sadly, as far as I can see the 'experts' in this field have failed to provide us with any useful data, so nobody really knows. Whether you say hypermobility is the same as EDS 3 is just a matter of words. Both of them are defined however you want to define them so some people can define them the same and others not. It doesn't make any difference to what we know about causes of joint pain.

 

[ahmo]

Mast cell expert Theo Theoharides' spoke earlier this week about mast cells and EDS. I haven't yet listened.

http://www.chronicpainpartners.com/...ders-by-dr-theoharis-theoharides-june-2-2015/

www.edsawareness.com

 

[Sushi]

All that we are interested in is whether widespread pain is causally related to hypermobility or whether, if they do occur together, it is an irrelevance. There are people with severe hypermobility who have dislocations, but I am unclear what that has to do with pain elsewhere.

Taking myself as an N = 1, I have hypermobility, EDS3 or whatever you want to call it, but my only pain is related to frequently getting "out of alignment" which causes muscle strain/pain and sometimes pressure on nerves. I regularly see an osteopath for manipulations to get my body back in line.

 

[charlie1]

Mast cell expert Theo Theoharides' spoke earlier this week about mast cells and EDS. I haven't yet listened.

MCAS and EDS and hypermobility...I will be reading / listening to that article as soon as I'm able!

I've always been hypermobile but I was still active...golfing horseback riding etc up to when I became ill 3 yrs ago which over time included time periods of MCAS and even longer time periods of POTS. During this time my hypermobility got worse and now causes frequent dislocations which in turn causes severe migraines (life long battle with headaches) until my body is re-aligned. If I could afford it, I would be seeing my PT daily as I can't put the bones in place. Especially problematic for me are my shoulders and elbows.

 

[WillowJ]

As JHS is a genetically determined disorder, the underlying defect, which is likely to be a mutation affecting one of the genes encoding one of the fibrous proteins of the connective tissue matrix (a collagen, elastin, fibrillin or tenascin), is determined at conception. Many people who inherit the defect remain symptom-free throughout their lives, whereas others may develop minimal musculoskeletal symptoms after unaccustomed exercise. The full panoply of complications does not appear all at once, but gradually over the first 2–3 decades of life.

No citations for this, though.

I always thought that connective tissue problems implied problems with muscles holding together (and with myself as an example, any really good PT/similar that I see will complain that my muscles are fragile, and may the blame connective tissue specifically), with the fascia around organs, with the blood vessels, and so on.... but variable because there can be different genetic problems that cause different problems in different areas.

From what I understand, there are thuoght to be more than one gene involved in the type(s) that don't yet have genes associated, and this causes complexity in the research.

This article seems to lack many citations. It's dated 2013.

Conclusion
Far from being a minor disorder occurring in essentially normal individuals, as is perceived by many of our colleagues even today, there is abundant evidence of a multisystemic hypermobility-related disorder that causes much suffering in the community. This is an emerging disease, which bears little resemblance to the joint hypermobility diseases originally described in the 1960s. To dismiss disparate symptoms as ‘illness behaviour’ is, in my view, wrong and does much disservice both to our patients and to our reputation as a profession. More often than not, JHS is still overlooked, misdiagnosed and hence untreated.21

Citation 21 is

1. Ross J, Grahame R. Joint hypermobility syndrome. BMJ 2011;342:c7167.

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There do seem to be more citations there.