What is HSD?
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.
What is h-EDS?
New Criteria for h-EDS:
Domain 1The 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 traumaDomain 3 The absence of any other underlying Heritable Disorder of Connective Tissue including other variants of EDS
Beighton Score for assessing joint hypermobility
- 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 then is BJHS?
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 All these names are considered outdated and their use should be discouraged.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)?
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).