Hypermobility

Hypermobility can be associated with widespread musculoskeletal pains although many people with hypermobile joints do not have musculoskeletal pain.

Referral Criteria

It is not necessary to refer patients with hypermobility to the Rheumatology service unless you are concerned about an alternative diagnosis. The diagnosis of hypermobility and Hypermobile Ehlers Danlos Syndrome (hEDS) should take place in primary care (see The Ehlers-Danlos Syndromes Toolkit).

There is no commissioned pathway for secondary care referral of hypermobility or suspected Hypermobile Ehlers Danlos Syndrome (hEDS)

Referral to the Pain Clinic may be appropriate if the patient's symptoms do not respond to the measures outlined in Primary Care Management.

Referral for Clinical Genetic testing is appropriate in specific sub-types of EDS (see management section)

Assessment

Signs and Symptoms

The diagnosis of hypermobility and Hypermobile Ehlers Danlos Syndrome (hEDS) should take place in primary care (see The Ehlers-Danlos Syndromes Toolkit).

History

  • Generalised joint pain
  • Back pain
  • Dislocations (uncommon)

Examination

Hypermobility can be diagnosed clinically by testing mobility of the hands, knees, elbows, back and wrists and calculating the modified Beighton Score (see link to image below).

Beighton Score Image

The Beighton score is calculated as follows:

  1. One point if while standing forward bending you can place palms on the ground with legs straight
  2. One point for each elbow that bends backwards
  3. One point for each knee that bends backwards
  4. One point for each thumb that touches the forearm when bent backwards
  5. One point for each little finger that bends backwards beyond 90 degrees.

Give 1 point for each of the manoeuvres you can do,up to a maximum of 9 points

If the score is greater than 4 and there is pain for longer than three months in four or more joints this (without synovitis*), this is consistent with hypermobility.

*Synovitis – described as soft rubbery swelling which bounces back when compressed.

Management

Primary Care Management

Education:

  • Explain that they have hypermobile joints and that sometimes this is associated with joint pain due to laxity of the ligaments and soft tissues. Reinforce that 'pain does not mean damage'. Explain that there is no cure but some treatments can help control pain.
  • Reassurance and de-medicalisation
  • Advice about keeping active: printable UK Chief Medical Officers' Guidelines available at UK physical activity guidelines (about halfway down the page)

Drugs:·

Physiotherapy:

  • Referral to physiotherapy is recommended for muscle strengthening exercises to improve joint stability.

Secondary Care Management

The Role of the Pain Team:·

  • Referral to the pain clinic may be appropriate if the patient's symptoms do not respond to the measures outlined in Primary Care Management.
  1. The Role of Clinical Genetics:·
    • Clinical genetics no longer routinely assess patients with hypermobile type Ehlers-Danlos syndrome (hEDS) or joint hypermobility syndrome (JHS). There is no genetic test available for hEDS as the genetic basis is still unknown. It does tend to run in families and so there is a possibility of other relatives experiencing similar problems. It is important to know that occasionally some rare and more serious genetic conditions, such as Marfan and Loeys-Dietz syndromes, or the vascular form of Ehlers-Danlos (vEDS, or EDS type 4), can present with hypermobility. A personal or family history of any of the following could indicate one of these:
  2. Dissection or aneurysm of a blood vessel including aorta.
  3. Organ rupture
  4. Significant skin hyperextensibility, true Cutis Laxa (not soft skin) or friability
  5. Facial milia
  6. Marfanoid habitus
  7. Pneumothorax
  8. Fragile sclerae
  9. Progressive scoliosis
  10. Dislocated lenses
  11. Detached retina
  12. Cleft palate or bifid uvula
  13. Craniosynostosis
  14. If your patient or their relatives have these, then please refer to Clinical Genetics..

Referral

Referral Criteria

It is not necessary to refer patients with hypermobility to the Rheumatology service unless you are concerned about an alternative diagnosis. The diagnosis of hypermobility and Hypermobile Ehlers Danlos Syndrome (hEDS) should take place in primary care; The Ehlers-Danlos Syndromes Toolkit

There is no commissioned pathway for secondary care referral of hypermobility or suspected Hypermobile Ehlers Danlos Syndrome (hEDS)

Referral to the Pain Clinic may be appropriate if the patient's symptoms do not respond to the measures outlined in Primary Care Management.

Referral for Clinical Genetic testing is appropriate in specific sub-types of EDS (see management section)


Supporting Information

GP Information

Beighton score

Beighton Score Image

Patient Information

Hypermobility Syndrome Association (HMSA)

The Ehlers-Danlos Syndromes Toolkit

UK physical activity guidelines

Versus Arthritis - Hypermobility

Pathway Group

This guideline has been signed off on behalf of the NHS Devon Clinical Commissioning Group.

Publication date: November 2019

 

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