Hypermobility

This page is currently under review – Nov 23

Hypermobile Ehler’s Danlos Syndrome (hEDS) is a condition characterised by hypermobile joints, widespread musculoskeletal pain and other symptoms. It is also known as Ehler’s Danlos Syndrome type III. There are many other types of EDS but most are very rare. Patients with these subtypes also have joint hypermobility but with prominent skin manifestations including fragile, paper-thin skin which scars easily. If these skin features are present, referral to clinical genetics would be indicated.

History

Generalised joint pains
Hypermobile joints
Back pain
Dislocations (uncommon)
Abdominal pain
Palpitations and faintness on standing
Easy bruising
Stress incontinence

Examination

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

Test	Score
Extend 5^th (little) finger by >90^o	1 point for each side
Extend thumb back to touch forearm or lie parallel to forearm	1 point for each side
Extend elbow by >10^o	1 point for each side
Extend knee by >10^o	1 point for each side
Bend over and touch floor with flat of hands with legs straight	1 point

A score of >4 is considered to represent joint hypermobility (the maximum score is 9).
Some patients also exhibit signs of fibromyalgia with multiple tender spots on palpation
There may be a late systolic murmur suggestive of mitral valve prolapse.
The pulse may rise and blood pressure fall on sitting up or standing up. This is known as postural orthostatic tachycardia syndrome (POTS)

It is not necessary to refer patients with hypermobility to the rheumatology service if there are no symptoms. If there is joint pain and generalised pain referral would be indicated if you are unsure about the diagnosis of thought there was another cause of the pain. Referral to the pain clinic may be appropriate if the patient’s symptoms don’t respond to the measures outlined in Primary Care Management.

Patients with prominent cardiac, GI and urinary symptoms may require evaluation by cardiology, gastroenterology or urology.

Management

Treatment is symptomatic.

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.
Explain that there is no cure but some treatments can help control pain.

Drugs: