What’s This Research About?

The variability and breadth of symptoms of hypermobility spectrum disorders have confounded research and medical fields. Some of the confusion in the medical field is due to the lack of conclusive genetic testing, inconsistent nomenclature, or lack of awareness about the condition. In clinics this population often ends up underserved without proper treatment and care. Because of this the authors tried to outline the physiology of the multitude of symptoms in JHS to help primary physicians better serve these patients to properly identify and manage the condition.

TITLE: Joint Hypermobility Syndrome: Recognizing a Commonly Overlooked Cause of Chronic Pain

ORIGINAL LINK

PUBLICATION: The American Journal of Medicine

DATE: 2017

AUTHORS : Bharat Kumar, Petar Lenart

Beighton score: A diagnostic scoring system that gives one point each for excess laxity on right and left: elbow, thumb, pinky, and knee. One point is given for forward fold with palms to floor. High score is 9, suggesting a high degree of joint laxity.

Brighton criteria: A set of clinical criteria and morphological features used for diagnosing Hypermobility Syndrome (HMS)  that includes a Beighton score of 4 or higher and joint pain in 4 or more joints for more than 3 months. It can also include abnormalities in the skin, marfanoid habitus and other signs of tissue laxity.

Dysautonomia: A malfunction of the autonomic nervous system that creates problems with regulating heart rate, blood pressure, digestion or temperature control.

Ehlers Danlos syndrome (EDS): A collection of heritable connective tissue disorders thought to alter the biology of collagen in the body (the most abundant protein), which can lead to multi-systemic symptoms, such as: hypermobile joints and soft, stretchy skin, easy bruising, easy wounding, poor wound healing and/or atrophic scarring.

Hypermobility type (hEDS): Within the Ehlers–Danlos spectrum, a similar subcategory of patients having similar clinical features as HMS but lacking a specific genetic profile.

Joint hypermobility syndrome (JHS)*:  a disorder of the connective tissue that can result in unstable joints, skin abnormalities, chronic pain, and fatigue. It can also include many other symptoms. The term “hypermobility” includes a an array of sub categories with a wide continuum of symptomatology. The broadest umbrella term is “hypermobility spectrum disorders”. The spectrum ranges from those who are high functioning (with the only symptom being generalized joint hypermobility), to people with so many symptoms they can be debilitated. The latter would most likely be someone diagnosed as Ehlers Danlos syndrome, hypermobility type (hEDS).

*This article was written before they came out with the newest nomenclature for the hypermobility spectrum disorders. The new nomenclature is below.

GJH: Generalized joint hypermobility
LJH: Localized joint hypermobility
PJH:  Peripheral joint hypermobility
G-HSD: Generalized hypermobility spectrum disorder
L-HSD: Localized hypermobility spectrum disorder
P-HSD: Peripheral hypermobility spectrum disorder
hEDS: Hypermobile Ehlers–Danlos syndrome (Castori et al. 2017)

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