Hypermobility and EDS, NHS referral and diagnosis

What hEDS and HSD are

Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders. Hypermobile EDS (hEDS) is the most common type. It causes joint hypermobility (unusually flexible joints), joint pain, frequent dislocations or subluxations (partial dislocations), stretchy or fragile skin, and a range of systemic symptoms.

Hypermobility spectrum disorder (HSD) is a related condition for people who have symptomatic joint hypermobility but do not meet the full criteria for hEDS. It is equally real and can cause significant disability.

hEDS and HSD commonly co-occur with POTS, MCAS, gastro-intestinal disorders, and chronic pain conditions.

The 2017 diagnostic criteria

The 2017 international criteria for hEDS require:

• Criterion 1: Generalised joint hypermobility. Positive Beighton score (see below), or evidence of hypermobility in multiple joints.
• Criterion 2: Two or more features from two categories:
  • Systemic manifestations: stretchy skin, easy bruising, hernias, organ prolapse, dental crowding.
  • Family history: one first-degree relative meeting hEDS criteria.
  • Musculoskeletal complications: chronic pain in two or more limbs, recurrent dislocations/subluxations, or soft tissue injuries.
• Criterion 3: Exclusion of other connective tissue disorders. This is where genetic testing comes in — to rule out other types of EDS and similar conditions.

All three criteria must be met for a diagnosis of hEDS. If only Criterion 1 is met, or if symptoms are present but criteria are not fully met, HSD may be the diagnosis.

The Beighton score

The Beighton score is a simple 9-point test of joint hypermobility:

• Can you bend your little finger back beyond 90 degrees? (1 point each side)
• Can you bend your thumb back to touch your forearm? (1 point each side)
• Can you hyperextend your elbow beyond 10 degrees? (1 point each side)
• Can you hyperextend your knee beyond 10 degrees? (1 point each side)
• Can you place your palms flat on the floor with knees straight? (1 point)

A score of 5+ is positive in adults. 4+ if aged 50–70. 3+ if over 70. If you score highly, document it with photos or ask your GP to record it formally.

The NHS referral pathway

Typical route:

• GP appointment. Describe joint pain, dislocations, skin features, and any family history. Ask for a Beighton score assessment.
• Initial tests. Blood tests to rule out inflammatory arthritis (ESR, CRP, rheumatoid factor, anti-CCP). X-rays or ultrasound if joint damage is suspected.
• Referral to rheumatology. Ask for referral to a rheumatologist with an interest in connective tissue disorders. Some areas have dedicated EDS or hypermobility clinics.
• Specialist assessment. The rheumatologist applies the full 2017 criteria, examines skin and joints, and takes a detailed family history.
• Genetics referral (if needed). If other EDS types need ruling out, or if there is a family history of vascular complications, you may be referred to clinical genetics.

Genetic testing

There is no genetic test for hEDS — the gene has not yet been identified. Genetic testing is used to:

• Rule out vascular EDS (VEDS) — this is critical if there is a family history of arterial rupture, bowel perforation, or uterine rupture at a young age. VEDS is life-threatening and needs different management.
• Rule out classical EDS, arthrochalasia EDS, and other rare types.
• Rule out similar conditions such as Marfan syndrome, Loeys-Dietz syndrome, and osteogenesis imperfecta.

A negative genetic test does not rule out hEDS — it rules out the other types. The diagnosis of hEDS remains clinical, based on the 2017 criteria.

If your GP dismisses you

hEDS and HSD are frequently dismissed as 'being bendy', 'growing pains', anxiety, or fibromyalgia. If this happens:

• Bring the 2017 criteria checklist and show how many features you meet.
• Document your Beighton score with photos or a GP-signed assessment.
• List associated symptoms: gastrointestinal issues, POTS-type symptoms, skin features, dental crowding — these help distinguish hEDS from simple hypermobility.
• Ask for rheumatology referral directly. You do not need the GP to make the diagnosis — you need them to refer.
• If referral is refused, use Right to Choose in England to pick a rheumatologist.

NHS management options

There is no cure for hEDS or HSD, but NHS management can significantly improve quality of life:

• Physiotherapy. Must be with a physio who understands hypermobility — standard strengthening can worsen symptoms if joints are not stabilised first. Look for a physio with EDS/hypermobility experience.
• Pain management. Chronic pain clinics, TENS, pacing strategies, and appropriate analgesia.
• Occupational therapy. Joint protection, splinting, ergonomic advice, and aids for daily living.
• Gastroenterology. For IBS-type symptoms, gastroparesis, or reflux common in hEDS.
• Cardiology / autonomic clinics. For POTS, orthostatic intolerance, and palpitations.
• Psychology. Chronic pain management, coping strategies, and support for the psychological impact of a chronic condition — not because the condition is "in your head."

Management is multidisciplinary. Ask your rheumatologist or GP for referrals to the relevant specialties.

Frequently asked questions