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 subclinical 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.

This is the first update 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:

2017 EDS International Classification

Original (now obsolete) text from October 2016:

(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).

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.

Meanwhile, here are 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.

11 Responses to The BRIGHTON Diagnostic Criteria

  1. Robb says:

    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 🙂

    • Jandroid says:

      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!

    • Jandroid says:

      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.

  2. Kristie says:

    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.

    • Jandroid says:

      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

  3. Rebecca Hammond says:

    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. 🙂

    • Jandroid says:

      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.

  4. Pat says:

    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

    • Jandroid says:

      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.

  5. Simon.p says:

    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.

    • Jandroid says:

      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

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