New Updates on HSD, JHS, and h-EDS (2017)

The new definitions, terminology and criteria for a better understanding of joint hypermobility disorders are here (2017). All the relevant links to articles in the Ehlers-Danlos Society website can be seen in one place: click here.

What is HSD?

Read the complete article “Hypermobility Disorders — An Update for Clinicians” By Alan Hakim, March 16, 2017 here.
What then is HSD? Read about Hypermobility Spectrum Disorder (HSD) here. Read a general overview on EDS vs HSD here.

Hypermobility spectrum disorders (HSD) are a group of conditions related to joint hypermobility (JH). HSD are intended to be diagnosed after other possible answers are excluded, such as any of the Ehlers-Danlos syndromes (EDS) including hypermobile EDS (hEDS). HSD, just like hEDS, can have significant effects on our health. HSD and hEDS can be equal in severity, but more importantly, both need similar management, validation, and care.

HSD and Ehlers Danlos Syndrome hypermobility type (h-EDS) are at present demarcated into two different diagnosis with considerable overlap of symptoms using a stricter diagnostic criteria for h-EDS. It does not mean HSD is less debilitating than h-EDS.
HSD being a spectrum now allows for a much larger range of people to get a diagnosis. I am hopeful that a lot of people dismissed as benign hypermobility will be now assessed using a different criteria. HSD itself is expanded by taking into consideration the localization of hypermobility. It also incorporates people who had joint hypermobility and have lost it with progressive age.
Excerpts: 
Joint hypermobility (JH) : The capability of joints to move beyond normal limits. JH can exist by itself or be a part of a more complex diagnosis. Those with joint hypermobility in a couple of joints (fewer than five) have localized joint hypermobility (LJH). Those of us with joint hypermobility in five or more joints are described as having generalized joint hypermobility (GJH).
There are other types of JH. Peripheral joint hypermobility is a form of that affects the hands and/or feet only. It is common in infants, toddlers, and children, in whom it is usually mild or has no serious effect. Another is proposed for older adults who have progressively lost JH, called historical joint hypermobility.
Joint hypermobility ranges from asymptomatic JH and GJH through to hEDS as part of the EDS. Along that continuum fall localized general spectrum disorder (L-HSD), peripheral hypermobility spectrum disorder (P-HSD), historical hypermobility spectrum disorder (H-HSD), and generalized hypermobility spectrum disorder (G-HSD). (From http://ehlers-danlos.com/what-is-hsd/) 

What is h-EDS?

Read about Ehlers-Danlos Syndrome (EDS), updated facts (2017) here.  Download the Ehlers Danlos Society brochure here. EDS hypermobility type or h-EDS is a subtype of EDS.
New Criteria for h-EDS: 
Domain 1
The presence of generalized joint hypermobility (based on the Beighton Score or 5-part questionnaire)
Domain 2
(A) – skin or fascia signs and/or pelvic floor concerns and/or Marfanoid features (having at least 5 of all the features mentioned
(B) a family history
(C) At least 1 of the following 3 presentations:
  • Musculoskeletal pain in 2 or more limbs recurring daily for at least 3 months, or
  • Widespread pain for ≥3 months, or
  • Recurrent joint dislocations in the absence of trauma –  3 or more atraumatic dislocations in the same joint, or atraumatic dislocations in 2 different joints occurring at different times, or medical confirmation of joint instability at 2 or more sites not related to trauma
Domain 3 The absence of any other underlying Heritable Disorder of Connective Tissue including other variants of EDS
Watch the 2017 EDS International Classification Webinar here. The webinar slides are here and the transcript here.

 

Beighton Score for assessing joint hypermobility

Central to all this is the assessment of joint hypermobility using the Beighton Score, read about it here and here.

 Beighton Score:

  • One point if while standing forward bending you can place palms on the ground with legs straight
  • One point for each elbow that bends backwards
  • One point for each knee that bends backwards
  • One point for each thumb that touches the forearm when bent backwards
  • One point for each little finger that bends backwards beyond 90 degrees.
What is then JHS? Joint Hypermobility Syndrome (JHS) has now become a historical term with the 2017 updates. Read about what used to be JHS here. If one was diagnosed as JHS, it is possible now they will fall under the category of HSD.

What then is BJHS?

It is my pet peeve, read about it here.

The new terminology within this spectrum updates and substitutes all previous terms used to define patients with JH but without a molecularly proved syndromic condition. Among these terms there are: Ehlers–Danlos syndrome type III, Ehlers–Danlos syndrome hypermobility type, hypermobility syndrome, joint hypermobility syndrome, and benign joint hypermobility syndrome. All these names are considered outdated and their use should be discouraged. 

Are clinicians discouraged? They were encouraged to drop the word benign in relation to hypermobility syndrome many years ago. Even before these 2017 updates, until last year, the accepted terminology by experts were: generalized hypermobility (GH), Joint Hypermobility Syndrome (JHS), h-EDS or EDS-HT (EDS hypermobility type) and JHS/h-EDS (when the diagnosing clinician found the demarcation difficult). But did that completely eradicate the use of the term benign joint hypermobility syndrome (BJHS)?

No.

Example:

Bisaralli, Rahul, et al. “Benign joint hypermobility syndrome: A case series.” Journal of Medical Society 31.1 (2017): 59.

Temizturk, Fatih, et al. “The frequency of benign joint hypermobility syndrome in patients undergoing inguinal hernia repair: a prospective study.” International Surgery (2016).

Sahin, Ebru, et al. “Impaired quality of life and functional status in patients with benign joint hypermobility syndrome.” MYOPAIN 23.1-2 (2015): 28-33.

Chelimsky, Gisela, et al. “Benign Joint Hypermobility Minimally Impacts Autonomic Abnormalities in Pediatric Subjects with Chronic Functional Pain Disorders.” The Journal of Pediatrics 177 (2016): 49-52.

Jacks, Stephanie K., and Matthew J. Zirwas. “Abnormal wound healing related to high-dose systemic corticosteroid therapy in a patient with Ehlers-Danlos syndrome benign hypermobility Type.” Cutis 98.4 (2016): E20. (This one is even more surprising!)

Kaur, Navjyot, et al. “Benign joint hypermobility syndrome with postural orthostatic tachycardia syndrome and acrocyanosis.” Medical Journal of Dr. DY Patil University 10.2 (2017): 175.

Çınar, Muhammet, et al. “Investigation of joint hypermobility in individuals with hyperbilirubinemia.” European Journal of Rheumatology 4.1 (2017): 36. (BJHS mentioned in body text)

Vogelgesang, M. D. “Recognizing, Diagnosing and Treating Benign Joint Hypermobility.” (2016).

videotogif_2017.03.23_15.52.20

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About Hypermobility Syndrome India/Viv

I an an Indian woman with Ehlers Danlos Syndrome Hypermobility Type (EDS-HT), also known as joint hypermobility syndrome. Although I have "suffered" from this disease all my life, I was diagnosed at 38 years, 2 years after my child was born. My work mainly involves being a mom to my beautiful and compassionate child. I also write and work from home in the field of science. I aim to raise awareness about EDS-HT, chronic pain, invisible illnesses, mental illnesses and invisible disabilities through my blogs. Viv (Latin root, meaning Life/ Alive) is my blogging pseudonym and alludes to my continued endeavor to rediscover my life and its meaning.
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