Skip to main content

Unfortunately we don't fully support your browser. If you have the option to, please upgrade to a newer version or use Mozilla Firefox, Microsoft Edge, Google Chrome, or Safari 14 or newer. If you are unable to, and need support, please send us your feedback.

We'd appreciate your feedback.Tell us what you think! opens in new tab/window

Elsevier
Publish with us
AI-Generated response:

What is Generalized Hypermobility Spectrum Disorder?

Answer generated on June 4, 2024

The AI-generated responses are for illustration purposes only and should not be used for medical purposes.

Generalized Hypermobility Spectrum Disorder (G-HSD) is a classification for individuals who exhibit significant joint hypermobility but do not meet the strict diagnostic criteria for Ehlers-Danlos Syndrome (EDS) or other connective tissue disorders.[1]

  • G-HSD validates the clinical status of patients with significant joint hypermobility and associated symptoms, ensuring they receive necessary medical services.[1]

  • It encompasses a range of symptomatic presentations, including pain, fatigue, joint instability, and interference with daily activities, which do not qualify as EDS.[1-2]

  • The Beighton scoring system is often used to assess joint hypermobility in these patients, with a high score indicating more pronounced hypermobility.[3]

  • Comorbid conditions such as orthostatic tachycardia syndrome (POTS) and orthostatic hypotension are common among individuals with G-HSD.[3]

  • Diagnosis is clinical, focusing on the exclusion of other heritable connective tissue disorders and assessing the extent of joint hypermobility and associated symptoms.[1][3]

Additional info

The diagnosis and management of G-HSD require a comprehensive approach that includes a detailed patient history and physical examination. It is crucial to differentiate G-HSD from other conditions like EDS and Marfan syndrome, which may present with similar symptoms but have different management and prognosis. The use of the Beighton score helps in quantifying joint hypermobility, which is a key diagnostic criterion. Additionally, recognizing and managing comorbid conditions such as cardiovascular issues, gastrointestinal problems, and emotional distress are essential for improving the quality of life of these patients. Clinicians should remain vigilant for the evolution of symptoms that might suggest a transition to a more defined connective tissue disorder over time.

References

Reference 1

1.

Elsevier ClinicalKey Clinical Overview

Diagnosis Historically, people with GJH were often diagnosed with a spectrum of conditions such as familial joint laxity or benign joint hypermobility syndrome (BJHS). Familial joint laxity largely explained a heritable condition with joint hypermobility with or without other musculoskeletal issues and is more descriptive than defining. Anecdotally, the age of presentation of daily joint pain or dysfunction in women is more often after the onset of menses. Many people with joint hypermobility, including GJH, are asymptomatic. There are many theories about why one hypermobile person has pain whereas another may not, but there has been little substantiation. Aside from EDS, most inherited disorders present with other findings that can help with diagnosis, as long as the clinician remains alert for such findings. In EDS, joint hypermobility is often the most prominent feature. EDS represents a group of 13 different but related heritable connective tissue disorders that have predominantly in common joint and skin manifestations ( Table 1 ). Joint hypermobility, and in particularly GJH, is common to all types and the Beighton scoring system is recommended to assess this in all types. Many patients with significant joint hypermobility (whether symptomatic or not) do not meet strict diagnostic criteria for any of the types of EDS or any other named condition but still are clearly outside the normal range of joint mobility and many have significant comorbidities. These patients should be classified as having hypermobility spectrum disorder (HSD) so as to validate their clinical status and help them receive necessary services They should not be labeled with EDS if they do not meet diagnostic criteria Many patients with joint hypermobility have other signs and symptoms, which help to differentiate between various connective tissue disorders such as Marfan, Stickler, or most of the types of EDS.

Reference 2

2.

Teran-Wodzinski P, Kumar A. Clinical Characteristics of Patients With Hypermobile Type Ehlers-Danlos Syndrome (hEDS) and Generalized Hypermobility Spectrum Disorders (G-Hsd): An Online Survey. Rheumatology International. 2023;43(10):1935-1945. doi:10.1007/s00296-023-05378-3.

Publish date: October 0, 2023.

To examine the perspective of individuals with Hypermobile Ehlers-Danlos Syndrome (hEDS) and Generalized Hypermobility Spectrum Disorder (G-HSD), needed to help identify priorities and improve the lives of people living with these conditions. We conducted an online survey between November 2021 and January 2023. Participants were recruited through the Ehlers-Danlos Society's Research Surveys website. We obtained 483 responses and 396 were analyzed. 80% of the survey's respondents were individuals with hEDS, 90% were females, 30% were between 21 and 30, and 76% lived in North America, with 85% of the participants living in North America indicated being White or European American. Participants reported exercising from none to less than three times per week, with no physical therapy treatment. Ninety-eight percent of... (truncated preview)

Reference 3

3.

Elsevier ClinicalKey Clinical Overview

Workup Focus exam on exclusion of organic causes of pain Assess for possible comorbid conditions Perform an assessment of joint hypermobility utilizing the most recent criteria for hEDS Generalized joint hypermobility with Beighton score of 6 or more out of a maximum of 9 points in children and adolescents Obtain orthostatic vital signs because orthostatic disorders (eg, postural orthostatic tachycardia syndrome [POTS]) are often comorbid with COPC Document blood pressure, pulse, and presence of any manifestations (eg, dizziness) after 10 minutes supine and at 1-, 3-, and 5-minute standing Abnormal heart rate values indicating orthostatic tachycardia may include Increase in heart rate by 40 or more beats per second in first 10 minutes of upright from supine position Abnormal blood pressure values indicating the presence of orthostatic hypotension may include Decrease in blood pressure exceeding 20 to 40 mm Hg systolic and 10 to 20 mm Hg diastolic within 3 minutes of standing from supine position and accompanied by manifestations (eg, light headedness, reflex tachycardia)

Follow up questions