The BRIGHTON Diagnostic Criteria
March 20, 2017: The following information is now completely obsolete since the brand new nosology and diagnostic criteria for all known forms of the (now plural) Ehlers-Danlos Syndromes was just released on March 15th, 2017, and the new diagnostic category for less visibly bendy or symptomatic patients was added called Hypermobility Spectrum Disorders, or HSDs.
This now catches all of those who aren’t quite “bendy” enough (as I lobbied so hard for below!) to meet the more stringent criteria for the hypermobile form of EDS (hEDS), the most common type still, and still lacking a known underlying genetic marker.
Alternatively, even if you’re bendy enough, even a 9 on the Beighton 9 pt scale, you may not tick enough required boxes under Criterion 2 here.
That is to say, you don’t have enough “issues with your tissues”. You may well be related to someone with an EDS however. So you would have an HSD. (I personally think Hypermobilitiy is a Red Herring now, myself, 2021.)
This is the first update to both the nosology and diagnostic criteria in 20 years~! (Count em, 20!) So please be patient with yourselves and your doctors as we ALL get slowly up to speed with the new information to be found here now:
https://ehlers-danlos.com/2017-eds-international-classification/
Though I highly recommend doctors check out either my Resources For Doctors page here, or the EDS Toolkit for Doctors published in 2018 here for a shorter “cliff notes” version to get up to speed more quickly:
https://gptoolkit.ehlers-danlos.org (yes, it just moved domains in January 2022, but lives on!)
However, I’m keeping this post for historical purposes, to help clear up any confusion arising from people reading other old literature and wondering which of the two names referred to what. And to share our history.
TL; DR, it’s:
Beighton 9 pt scale or score – which was part of the Brighton diagnostic criteria before 2017
Brighton Diagnostic criteria (described below) which are now obsolete 2017 which were used by some to diagnose hypermobile type EDS as well as JHS and HMS in the past. (Prior to 2017)
Yes, the names were SO close that almost no one could keep them straight!
Original (Now Obsolete) Text from October 2016 clarifying the two
(I am leaving here for historical purposes and reference only. PLEASE SEE THE UPDATED CRITERIA IN THE LINKS ABOVE TY! JG April 2017)
As I’ve mentioned elsewhere, it is best to see a medical geneticist whenever possible to get diagnosed with any form of Ehlers-Danlos Syndrome so as to rule out any other Heritable Disorders of Connective Tissue (especially Marfans, Sticklers and OI), and also the more rare and dangerous forms of EDS if suspected, especially Vascular.
But the most common type by far, Hypermobile EDS or hEDS (formerly called EDS III as well as HMS, JHS and BJHS elsewhere) has no single tissue marker identified yet (except for the rare autosomal-recessive Tenascin X variant) and still must be diagnosed clinically, that is, through careful informed physical examination and thorough family history (where available) using the Brighton Diagnostic Criteria (spelled with an “r” and not to be confused with the Beighton 9 pt Hypermobility Scale spelled with an “e” which is just one misleading part of it).
The Hypermobility Syndrome Association of the UK still intends the Brighton Criteria only for diagnosing “JHS” or Joint Hypermobility Syndrome. But many leading US doctors, Dr Jaime Bravo in Chile, and leading UK rheumatologist Professor Rodney Grahame believe JHS, BJHS and HMS should all just be called hEDS now. (See “The Lack of Clinical Distinction Between…” by Tinkle, Grahame et al on p. 17 of the Autumn 2010 EDNF Loose Connections). [Edit 2019: this is now obsoleted by these.]
However, I’m posting the Brighton Diagnostic Criteria shown below from the HMSA website as they are presented nicely there to get you acquainted as they are the only generally recognized clinical method available for diagnosing HEDS at this time as far as I know.
I have italicized the seven minor criteria that do not require you to be bendy (flexible) at all (i.e, you can score a zero on the Beighton 9 pt scale even if you are under age 50!), and I’ve bolded the four which have nothing to do with your joints! This latter is why a lot of US rheumatologists and even some geneticists still fail to diagnose us as they keep only looking for grossly bendy patients or stretchy skin which you do NOT have to have! (You can have thin skin that is soft or tears easily instead.)
Further, I’m pleased to see Hamonet at al agree with me on the inefficacy of the Beighton 9pt scale in this paper from 2015 where they state:
“A Beighton score equal to or greater than 4 is suggestive of hypermobility. However this test is often misleading or poorly applied; it considers only a small number of joints [11], it does not take into account pain or muscle contractures, and therefore wrongly rules out a large number of patients. Thus a negative score is not incompatible with a positive diagnosis, especially in adults [7]”
I developed a totally unofficial Reverse BRIGHTON Criteria slide showing them in reverse for this very reason, to help de-emphasize the hypermobility aspect that keeps tripping everyone up (both doctors and patients) that I first shared in this post about not being rare. I like to say “we got Bright in Brighton, England” to keep them straight, smile.
The Original Brighton Diagnostic Criteria (not scale!) (via the HMSA of the UK)
Revised diagnostic criteria for the Joint Hypermobility Syndrome (JHS)
Major Criteria
- A Beighton score of 4/9 or greater (either currently or historically)
- Arthralgia for longer than 3 months in 4 or more joints
Minor Criteria
- A Beighton score of 1, 2 or 3/9 (0, 1, 2 or 3 if aged 50+)
- Arthralgia (> 3 months) in one to three joints or back pain (> 3 months), spondylosis, spondylolysis/spondylolisthesis.
- Dislocation/subluxation in more than one joint, or in one joint on more than one occasion.
- Soft tissue rheumatism. > 3 lesions (e.g. epicondylitis, tenosynovitis, bursitis).
- Marfanoid habitus (tall, slim, span/height ratio >1.03, upper: lower segment ratio less than 0.89, arachnodactyly [positive Steinberg/wrist signs].
- Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring.
- Eye signs: drooping eyelids or myopia or antimongoloid slant. [JG – aka almond-shaped eyes]
- Varicose veins or hernia or uterine/rectal prolapse.
JHS is diagnosed in the presence two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative.
JHS is excluded by presence of Marfan or Ehlers-Danlos syndromes. Criteria Major 1 and Minor 1 are mutually exclusive as are Major 2 and Minor 2.
NB italics are mine, to highlight the seven minor criteria you can still pass and use to diagnose without scoring at all on the Beighton 9 pt Hypermobility (bendy) Scale. (That is how you can be a 0 and still pass!) I also highlighted four criteria in bold that have nothing to do with your joints! If you pass any 4 minor criteria, or any 2 minor plus either major, or both major criteria you pass. (You also pass if you have a known 1st degree relative and any 2 minor criteria.) That’s a lot of ways to pass without being bendy!
Also, as stated by the HMSA rather dryly at the bottom, the first two Minor criteria are essentially a restatement of the two Major criteria, but with a much lower threshold. So they are mutually exclusive.
I.e, if you pass just one of the Major criteria, you cannot also count whichever of the first two minor criteria that matches, since obviously you have already surpassed its lower threshold by passing it as a major criteria. You need to pass other non-matching minor criteria in addition to whichever major criteria you passed. (Sorry if that’s confusing, but there you are.)
I dearly hope this helps some more of you to suspect and hopefully get diagnosed with Hypermobile EDS where appropriate with the help of your doctors. I will go to my grave trying to get people to stop telling me their “number” from the Beighton 9pt scale only!
As I’ve hopefully made clear enough here, you can be a zero and still have hEDS! You don’t get a “score” from the Brighton Criteria either, you get a criteria combo (e.g. “2 major”, or “1 major and 3 minor”, or “5 minor:”), so anyone who says “but I’m just a 3” on the Beighton Hypermobility Scale only is doing it wrong.
I’ve lost track of the number of patients who’ve come to me in great distress after being quickly dismissed by uninformed doctors who ONLY look at the Beighton 9 pt scale and not the rest of the Brighton Diagnostic Criteria and stop short and fail to diagnose them despite obvious and even life-threatening extra-articular (non-joint related) issues.
Thank you for reading this far and trying to get it right with me! I’m also going to go to my grave lobbying for a name change – they differ by only one letter! Is it any wonder everyone gets confused??~! Obviously I ultimately hope they come up with more of the tissue markers and a better naming system and soon, but meanwhile this is the best we have. You can see me attempting poorly to explain this (while in great pain) in person at our not so “mini-conference” in Oregon in 2013.
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Thanks to your post I know have not anymore doubts about this thing of the beighton score/brighton criteria: I score 0 in Beighton, but 1 major and 2 minor in Brighton’s criteria.
Do you you now which countries in Europe do use brighton’s criteria to diagnos esd?
I live in Italy and I will soon go to the Esd Centre in my city, Milan, but I am kind of afraid they will not confirm my HMS because I score 0 at beighton’s test! What if they don’t consider brighton’s criteria? /:
Is this method already used by every doctor specialized in esd??? I hope so… let me know what you think!
Thank you 🙂
Wonderful! So glad to know I was able to clear up the confusion for you -it’s no help that the two names are off by one lousy letter, and doctors only promote the very misleading Beighton 9 pt scale so far.
That said, I’m eager to hear what came out of the Hypermobility discussions at the International EDS Symposium in New York City last week (May 3-6, 2016) – I may have/get to change this post (or even delete it!) a bunch soon… stay tuned!
Oh, and sorry, but I do not know what countries use/do what regarding the Brighton DX Criteria sorry. I would just arm yourself with the articles from my website to use to approach any doctor you see anywhere, even in the US. What I shared in this post is very poorly known all over yet, even if it’s about to change already (May 2016) too.
Best of luck to you.
Thank you for this article. I’m in the process of trying to get EDS diagnosed in myself, after dealing with its symptoms through over 25 years of nothing being found to explain them, and I’ll be taking this information with me to my GP appointment tomorrow.
You’re quite welcome. It shouldn’t even take 25 weeks much less 25 years, so I’m glad I can help a little. (It also took me over 25 years to be properly diagnosed finally and only after becoming “grossly” visible, i.e, “clinical” after falling apart head to toe. It shouldn’t take that either to get diagnosed!)
Good luck,
Jan
Thank you for all your work on this site. I don’t even know how I came across it but, I’m a nurse and someone who likely has EDS (awaiting genetics referral) and it’s hard to find such comprehensive, clear info in one place. 🙂
Aw thanks – that is my dearest wish, to help people wade through the miasma of often old, outdated and even conflicting information out there. So I’m glad you felt it so helpful. Good luck.
I can relate to so much of this. What kind of doctor do I see and what do I ask for to be tested to find out if I have all of this? I am confirmed FM,RA,Lupus and more
Technically any doctor that is willing and savvy enough can diagnose EDS, but… I recommend seeing a medical geneticist whenever possible for several reasons. A) They are better trained at gathering more complete family medical history that will inform the diagnosis, B) they can rule out similar heritable disorders of connective tissue, and C) sadly, many other doctors including some specialists you may need will not accept a diagnosis from any other kind of doctor. (This is really too bad IMHO, but that’s the state of things currently, sorry.)
I recommend reading this post to prepare as well as my Resources page to find good articles for yourself and your doctor(s), and gather as much family history as you can even if you think it’s not related (you’d be surprised!) and lobby lobby lobby!
http://ohtwist.com/about-eds/diagnosing-eds/
We have to be the poodle on the pantleg of our doctors sometimes. But the squeaky wheel eventually gets oiled. Finding a local support group via http://chronicpainpartners.com may help too. HTH. Good luck.
very interesting site. Thank you for posting all the information. I need a DX for my 8 year old son who is struggling to keep up in mainstream school. It seems everyone keeps sending us to other services and no one has any answers so i am arming myself with all the knowledge I can as I’m sure he has hypermobility. so once again thank you for all the time you have spent learning and sharing this information.
Aw thank you for your kind words, I’m really glad my site is informative and helpful. That said, let me make really clear that the information above on this page, aka the Brighton Diagnostic Criteria are now completely obsoleted by the new hEDS diagnostic criteria which you can now find here:
https://ehlers-danlos.com/wp-content/uploads/hEDSvHSD.pdf
That said, if your son (and you) are not very bendy, that is, you do not pass the Beighton (with an “e”) 9 pt hypermobility scale for your age and sex as outlined in the above link, you may STILL have a form of the new diagnostic category called Hypermobility Spectrum Disorders they just hatched for others who still show signs of a connective tissue disorder, but are not “bendy” enough for the newly tightened hEDS criteria.
They now realize there is clearly STILL something going on, you still need and deserve medical attention and management (or SHOULD!), but it just doesn’t meet the new bar of entry to the forms of EDS. Make sure to take a thorough family history though and rule out any and all other suspected forms of more rare CTDs like Marfan, Stickler, OI and the rare forms of the EDS. More on this new category is here now:
https://ehlers-danlos.com/what-is-hsd/
This information and these criteria are BRAND NEW as of last month, March 15th, 2017, the first update in 20 years~! So please be patient with your doctors and nurses who are most likely NOT yet aware of this change. Print out what you can to take to them, and refer them (and yourself) to this page here for more information:
https://ehlers-danlos.com/2017-eds-international-classification/
I hope that helps, and best of luck to you! Hypermobility Spectrum Disorders are not rare, just rarely diagnosed yet. x
The new criteria are complete TRASH, and we’re created despite the overall vote to avoid changing hEDS criteria…the criteria were changed (to new criteria with poor to NO scientific reasoning (as many of the criteria are not specific to EDS, or even CTDs in general!) because the executive director, Lara Bloom, WANTED them changed. Not surprisingly, she still qualifies as “having EDS,” while many of us, too ill to work (and DEFINITELY too ill to make a narcissistic film about walking a marathon!) now no longer qualify!
These criteria will be torn apart by peer reviewed, and I cannot BELIEVE that the Journal was even willing to publish them. As well, these are CLASSIFICATION (read: research-level) criteria…DIAGNOSTIC criteria were never provided, and the ED Society is trying to act as if that’s normal (it’s not).
Try researching the backstories on Lara’s work, on the name changes in charities (such as EDS UK to THE EDS UK, etc), the missing funds in some of their publicly viewable financials (including one year when over 36k went UNACCOUNTED for!). There is a MAJOR conflict of interest in having a patient THAT IS NOT A DOCTOR listed as one of the co-authors on such a paper…there is also a conflict of interest with Dr. Francomano being on the Board, and getting money for her center, and the dubious “CINK” study.
Oh! Nearly forgot the conflict of interest with needing to use the genetic company that they are working with to become a part of the “registry”…the same genetic testing company that helped to FUND the symposium.
The ED Society is, in my eyes and the eyes of many others, not to be trusted…and even if it takes a while, their falsehoods and MASSIVE conflicts of interest will be exposed. In the meantime, many will be denied a NEW dx, or LOSE an old one. All so that TEDS can make EDS seem more “rare,” to gain rare disease funding…after YEARS of stating that EDS was widely underdiagnosed!!! Suddenly they come up with completely random criteria, made solely to make the diagnosis MUCH more rare, to get more funding, and to increase their own prestige – if the widespread nature of EDS was truly known, they would no longer have a choke-hold on all things EDS!!
Does no one here dare to even QUESTION why a SINGLE CHARITY has so much power in the lives of those with EDS? I know that I, for one, will refuse to sit down, remain quiet, and accept the head-patting dx of HSD, told that I should “still be treated the same,” despite the name recognition of EDS being the ONLY thing that finally got doctors to listen to me. I refuse to lose my EDS dx simply because it is a means for the Society to make “actual” EDS, hEDS particularly, 85-90% more rare, based on the words of some of their OWN doctors.
I’m waiting to see them implode from their wrongdoings and misappropriation of funds, of their desire to push an image of EDS as “dazzling,” and something that Lara (again, NOT A DOCTOR), to say on tv was helped with “exercise and supplements. And yet the greater part of the EDS community still does not see that the Society is nothing but a wolf in sheep’s clothing, corporatizing EDS for their own prestige and financial gain, while patients suffer, and have an even MORE difficult time of getting a dx than EVER before.
I’m absolutely disgusted by their actions, which completely fly in the face of all that they said at their last conference (as well as all they’ve said BEFORE that, about under-diagnosis, stiffness as an EDSer ages, etc.)…at a point AFTER this was treachery was decided upon, but before it was released.
I will never donate a single dollar to that organization again…and I hope that others don’t, as well. It may take a while, but more and more people are coming to see the light about how this “charity” organization works.
Wow Caylee, this is all news to me if true. I’m sorry you are feeling so upset by The EDS and the new criteria. I honestly hadn’t heard anything of this sort, but as I don’t know, I won’t say either way – I’ll just allow for the possibility to exist in the absence of seeing hard evidence yet.
That said, I am Exhibit A of someone who is quite seriously affected/afflicted by this lousy condition, whatever they want to call it, yet no longer meet the newly tightened dx criteria for hEDS with which I was diagnosed by a geneticist in Portland, Oregon, USA using the (now obsolete) Brighton dx criteria above along with thorough family history I brought in on a spreadsheet and a very thorough physical examination of me. (Trust me, they needed a spatula to get me out of the wheelchair at the time.)
Here’s the thing: I care less what they want to call what I have. Like Pluto, who didn’t change a spot nor stop orbiting when they reclassified it to a dwarf planet back in 2006, I haven’t changed a spot (well, okay I’ve stiffened greatly with age, early arthritis and knotty muscles from holding my loose skelly together all these years), and am in as much or more pain as ever before. I’m still orbiting the same hypermobile sun, the same way as always!
But guess what? I’m still receiving the treatment I need, and getting my pain managed and will. Just like my elderly aunty Sr. Kathleen Groh, (yes, a rare Catholic nun, and dying breed) back east at almost 93. She was never diagnosed, has as many issues as myself or more, similar history (fell apart in her 40’s), but has always gotten the care she needs without ever getting diagnosed with EDS or HSD or anything else. They just kept patching her up, and do, as issues arise. She’s one of the happiest people I know despite it.
I.e, while I do want and hope to get this condition much better recognized, I’m playing the “long game”, and willing to lose this “battle” (my current diagnosis technically of hEDS should that come to pass ever) in order to win the “war” (awareness that hypermobility is not so benign and can widely vary and is far from rare) whatever we want to call it. And, further, for what it’s worth, I know people WITH a full diagnosis who clearly meet the new criteria who STILL get mistreated and dismissed… sadly I find/feel that western allopathic medicine seems to select for narcissists and bullies, truly. It’s bad for everyone, including the doctors who are taught to ignore and dismiss THEIR OWN symptoms and issues while going through the meat grinder called medical school today. Sadly, they tend to pass this on (along with lots of B&W all or nothing rigid thinking) to their patients, not allowing themselves to either a) say “I don’t know” (which would be fine!) or b) ever admit they are wrong, or might be.
The system needs to change for everyone. Meanwhile, pick your battles. If you have an issue with The EDS, then take them on, and good luck. I don’t currently, as I am happy with my current status: I’ve stopped letting what others label me or think define me/my happiness. I will take this comment with a grain of salt, and keep an open mind. But I will withhold judgment until and unless I see evidence of same for myself. I frankly have bigger fish to fry. (I have a book to get out and I want to go back to work.) But thank you for sharing your insight. I will keep it in mind.
Meawnwhile, as I shared in this post, validate yourself:
http://ohtwist.com/2017/03/21/validate-yourself/
And know that I support any and all people who suspect a form of either EDS or HSD, or whatever else we end up calling it. I think molecular testing will eventually catch up one day, maybe even sooner than later, and we may well be vindicated, and they’ll realize it was the same thing (or something awfully close) all along. (And it won’t be one single SNP, either, I’m pretty sure.) But keep the faith, meanwhile, and believe in yourself and trust your gut and take good care of yourself for the long game. Think marathon, not sprint… Peace, Jan
My daughter was diagmosed hypermobile age 2 she needed pedro boots to get her walking now aged 9 she has awful pains in her legs she scored 0 on beighton so is not getting any further treatment. Her sister is awaiting assesment imward feet rolling ankles and she too has scored 0 but shes also being assessed for autism has reynauds and bowel problems. My cousin has eds and the doctors won’t even consider it
I’m so sorry for this development Kelly. That said, let me alert you to the brand new diagnostic category they just recognized last year, in March 2017 called Hypermobility Spectrum Disorders, for those who are either a) not bendy enough (ever or any more) to pass the Beighton 9 pt scale or b) if bendy, don’t have enough “issues with your tissues” (trouble) to tick enough boxes to pass the newly tightened hEDS criteria. Check it out here:
http://ohtwist.com/hypermobility-spectrum-disorders
I want to be clear these Brighton Diagnostic criteria shown on THIS page (above) are now obsolete, and replaced by these here:
https://ehlers-danlos.com/wp-content/uploads/hEDSvHSD.pdf
So even if they don’t recognize her/you all as having a form of EDS anymore, they can and should rule out other issues/conditions, and then suspect and consider a diagnosis of a form of Hypermobility Spectrum Disorder at least.
Please trust your gut, and mother’s intuition, and think marathon, not sprint. And keep persisting. You can and will eventually get her recognized and diagnosed.
The new diagnostic criteria are blatantly framed to exclude because of the directors of the EDS society fetishizing their own status of having a ‘rare’ disease.
Even when asked for the difference between hEDS and HDS, they cannot give any real dofference- and in fact state that the two “have the same symptoms at the same severity.”
And yet, they know full well that excluding patients from having the diagnosis of EDS- which is KNOWN as a severe disease- is going to worsen the care of thousands- or rather, hundreds of thousands- of patients.
I quite agree. And am composing my chapter on EDS for my book with this very much in mind. I first fell for them as “the authority” on all things EDS. But have since come to realize that while helpful on many fronts, and the Biggest Voice, so far, it’s really just an egotistic extension of the leader, who needs to feel special (rare) and to heck with everyone else, to whom we’re just paying lip service.
Thanks for writing in.