hEDS criteria 2017, for the newly diagnosed

Hypermobile Ehlers-Danlos syndrome is the most common type of EDS and the only subtype without a confirmed gene. Diagnosis is clinical. That means a doctor walks through a checklist and decides whether you meet criteria, with no genetic test that confirms or denies it. The current checklist is the 2017 international classification.

If you have been newly diagnosed and you are trying to figure out what your own paperwork says, this is the structure you are looking at.

Three criteria, all required

The 2017 framework has three criteria. All three are required. Missing one means the formal label is not hEDS, even if the picture looks close.

Criterion 1 is generalized joint hypermobility. The Beighton score is the bedside measure. The cutoff depends on age. Six out of nine for prepubertal, five for adults under 50, and four for adults 50 and over. The Beighton score does not capture every joint, so the criterion includes a five-point questionnaire for adults whose Beighton has dropped with age. A "yes" on two or more of the five items can substitute.

Criterion 2 is a set of features clustered into A, B, and C. A is systemic features such as soft skin, mild skin hyperextensibility, atrophic scarring at specific sites, hernia, pelvic floor prolapse, dental crowding, arachnodactyly, arm-span-to-height ratio, mitral valve prolapse, and aortic root dilation. B is a positive family history. C is musculoskeletal complications such as chronic pain, dislocations, and a positive history of joint instability. The diagnosis requires at least two of A, B, and C, with specific item counts inside each. Your geneticist or rheumatologist will mark these explicitly on the form.

Criterion 3 is exclusions. Other connective tissue disorders that look similar must be ruled out. Marfan, Loeys-Dietz, classical EDS, vascular EDS, and a list of others. The exclusions matter for your safety. Vascular EDS in particular has different surgical and obstetric implications. If you have features that suggest a different EDS subtype or a related disorder, the workup goes there first.

Outside the criteria

The criteria are clinical. They use the Beighton score, the feature cluster, and the exclusion list. Genetic tests, imaging, and specific comorbidities sit outside the framework. POTS, gastrointestinal dysmotility, MCAS, and chronic fatigue often travel with hEDS; they are common and real; they are managed alongside hEDS rather than as part of it.

Age at diagnosis sits outside the framework too. Adults often arrive at the workup after years of partial answers. That is normal. The clinical history goes back to childhood signs even when the diagnosis lands at 40.

How to read your own assessment

When you get the workup, the note usually has a section that goes through the three criteria explicitly. If it does not, ask. The Beighton score should be there as a number, with the joints scored. The 2A, 2B, 2C breakdown should be there, with the specific items checked. The exclusions should be there. If the conclusion is hEDS, those three sections explain why.

If the conclusion is hypermobility spectrum disorder rather than hEDS, the difference is usually that you do not meet the full criterion 2 cluster. Hypermobility spectrum disorder is a real diagnosis with similar clinical implications. Management is similar. The label is different because the criteria draw a line.

What changes after the diagnosis

Practically, three things. Physical therapy oriented to joint stability and proprioception is the primary intervention. Activity modification and pacing reduce flares. Comorbidity workups for POTS, gastrointestinal symptoms, and pain follow whatever symptoms are loudest.