The Ehlers-Danlos syndromes are associated with many comorbidities, including rheumatic diseases.

The article Ehlers-Danlos syndrome hypermobility type is associated with rheumatic diseases seems to be the most recent report on the associations. While relevant and important, the article was published in January 2017, a few months before the updated nosology was published, so it’s relationship to hypermobile EDS as opposed to EDS hypermobility type (for more about this distinction, please see Hypermobility – A History) is unclear.

In fact, the diagnostic criteria used to identify the “HEDS” patients for the study was actually not the Villefranche criteria created to diagnose HEDS, but rather the Brighton criteria, which were originally established to identify JHS patients, not HEDS patients at all. The authors were working under the old way of thinking, that JHS and HEDS were essentially two names for the same disease, when the new nosology, published in 2017 after the study was completed, indicates that they are in fact NOT the same, and not only significantly changed the diagnostic criteria for identifying the re-dubbed hypermobile EDS (hEDS), but also essentially replaced JHS with Hypermobility Spectrum Disorders, which are not diagnosed using the Brighton criteria.

Despite this flaw in terminology, however, the article is still relevant to discussions of hypermobility and rheumatic comorbidities, and their findings are significant. They point out that

“Perhaps due to a lack of gravitas surrounding the HEDS diagnosis, management of the disease varies among practitioners, and a clinical workup does not often extend beyond the joint and skin examination.”

By examining medical records, they were able to identify 379 patients in their database diagnosed with HEDS (JHS), then divided those patients into 3 different groups depending on the level of additional workup the patients received: no workup (240 patients), limited work up (51 patients), and comprehensive workup (88 patients).

They found that

“HEDS patients with back pain, joint pain, and joint laxity were more likely to be diagnosed with at least one rheumatological condition when they received comprehensive serological and radiographic workup for their musculoskeletal complaints in addition to a physical examination (67.1%).”

“Even a limited workup revealed significantly more rheumatological conditions among HEDS patients than a simple physical exam (i.e. NWU [no work up]) did.”

“Therefore, a more comprehensive workup tended to reveal a more complex clinical picture than a purely physical examination in these patients: nearly 15% of patients who had a comprehensive workup were diagnosed with three or four rheumatological conditions…. Our findings indicate that a more complex clinical picture may emerge from complaints of back pain, joint pain, and joint laxity following comprehensive serological studies (with or without radiographic studies) than a “straightforward” HEDS diagnosis.”

So basically, if the doctors bothered to look for anything else, the odds were high that they would find something, and if they were comprehensive, they tended to find quite a bit. Which is why the authors state they, “strongly support the adoption of comprehensive workup for all HEDS patients as a standard clinical practice.”

List of Rheumatic Conditions

These are the rheumatic conditions listed as “significantly more prevalent in the CWU HEDS population than in the general population of the US” in the article Ehlers-Danlos syndrome hypermobility type is associated with rheumatic diseases:

  • club foot
  • hereditary angioedema
  • primary hypogammaglobulemia
  • fibromyalgia
  • erythromelaglia
  • psoriasis
  • psoriatic arthritis (PsA)
  • ankylosing spondylitis (AS)
  • rheumatoid arthritis (RA)
  • inflammatory eye disease
  • autoimmune thyroiditis
  • systemic lupus erythematosus (SLE)
  • Crohn’s disease
  • pernicious anemia
  • TNF Receptor-Associated Periodic Fever Syndrome (TRAPS)

Sources

Rodgers KR, Gui J, Dinulos MBP, Chou RC. Ehlers-Danlos syndrome hypermobility type is associated with rheumatic diseases. Scientific Reports. 2017;7:39636. doi:10.1038/srep39636.