Who: Dr Helen Cohen, Consultant - Rheumatology and Chronic Pain (Royal National Orthopaedic Hospital, UK)
What: Ehlers-Danlos Society Learning Conference - Patient Day
Where: Macquarie University, Sydney
When: 7 December 2018
These are my notes from Dr Cohen's talk. I have tried to be as true as possible to what was said at the conference - please excuse any errors.
PRE-2017 THERE WAS A LOT OF CONFUSION:
Lack of diagnostic tests: Connective tissue diseases are different than many other diseases that have lab work or imaging that can be done to help determine the diagnosis. Since there is no nice, diagnostic test for the spectrum of hypermobility, the development of criteria became necessary.
Name of diagnosis confusion: Pre-2017, various names were used: Joint Hypermobility Syndrome, Benign Hypermobility (although it never really felt 'benign' to patients!), EDS Type III/EDS Hypermobility Type, Hereditary Connective Tissue Disorder
Diagnostic criteria confusion: used the Brighton Criteria or the Villefranche Criteria?
IN 2017 THE CONFUSION WAS CLEARED UP (mostly - until it changes again!):
- Names of diagnoses: Those four diagnoses became two (the old names above are now out-of-date and no longer used)
For a generalised overview look at HYPERMOBILITY AND THE EHLERS-DANLOS SYNDROMES (EDS) - Quick Facts (on this website).
Links below are to authoritative articles on these subjects:
Hypermobility Spectrum Disorder (HSD): subtypes= Localised, Peripheral, Generalised, Historical
- Diagnostic criteria:
- Spectrum: hEDS and HSD are both on a large spectrum of severity and range of symptoms
The 'label' doesn't define severity. One is not 'worse' than the other and a person with hEDS is not necessarily sicker or in more pain than someone with HSD.
Both hEDS and HSD can be very painful (but not for everyone!). Since our bodies are not designed to handle pain 24/7, the pain receptor system can get 'wound up' = central sensitisation.
Rheumatologists should already be used to the idea of diseases on a spectrum - eg, Palindromic rheumatism, Sero-negative RA, Sero-positive RA, Felty's syndrome
-Why draw the line? Differentiating between the two is potentially useful in research and in some clinical situations. It might make a difference in health or travel insurance. In general though, as treatment is based on an individual's symptoms, this is less important.
-Co-morbidities: hEDS, and to a much lesser extent HSD, can have co-morbidities (associated conditions, rather than caused by hEDS or HSD)
-Be careful of the overlaps: It is possible to have one or two hypermobile joints and not have hEDS or HSD - eg, someone with Fibromyalgia might have one bendy thumb - but they don't satisfy the criteria for generalised hypermobility. Generalised joint hypermobility is what really best characterises HSD and EDS.