Functional gastrointestinal disorders are increased in joint hypermobility-related disorders with concomitant postural orthostatic tachycardia syndrome

Neurogastroenterol Motil. 2020 Dec;32(12):e13975. doi: 10.1111/nmo.13975. Epub 2020 Aug 16.


Background: Individuals with hypermobility spectrum disorders/hypermobile Ehlers-Danlos syndrome (HSD/hEDS) frequently fulfill criteria for Rome IV functional gastrointestinal disorders (FGIDs). Postural orthostatic tachycardia syndrome (POTS) is also commonly reported in HSD/hEDS and may impact on co-morbidity with and severity of FGIDs, although this remains to be studied. We determined the impact of concomitant POTS and HSD/hEDS on their association with Rome IV FGIDs.

Methods: With the help of the charity organization Ehlers-Danlos Support UK, an online cross-sectional health survey was completed by individuals with HSD/hEDS. The survey enquired for (a) self-reported doctor diagnosis of POTS, chronic fatigue syndrome, and fibromyalgia, (b) the presence and symptom frequency of Rome IV FGIDs, and (c) anxiety and depression scores.

Key results: Of 616 subjects with HSD/hEDS, 37.5% reported a doctor diagnosis of POTS. POTS-positive individuals were significantly younger than POTS-negative subjects (37 vs 40 years, P = 0.002), more likely to report chronic fatigue syndrome (44% vs 31%, P < 0.0001), and showed a trend toward increased prevalence of fibromyalgia (44% vs 37%, P = 0.06) and higher depression score (P = 0.07). POTS-positive subjects were also more likely to fulfill criteria for Rome IV FGIDs across various organ domains and experienced both upper and lower gastrointestinal symptoms significantly more frequently. The increased associations for FGIDs and GI symptom frequency remained unchanged in HSD/hEDS subjects with POTS following adjustments for age, chronic fatigue syndrome, fibromyalgia, and depression scores.

Conclusions and inferences: The high FGID burden in HSD/hEDS is further amplified in the presence of POTS. Future studies should elucidate the mechanism by which POTS arises in HSD/hEDS and is associated with increased GI symptoms.

Keywords: functional gastrointestinal disorders; joint hypermobility; postural orthostatic tachycardia syndrome.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Cross-Sectional Studies
  • Ehlers-Danlos Syndrome / diagnosis
  • Ehlers-Danlos Syndrome / epidemiology*
  • Ehlers-Danlos Syndrome / physiopathology*
  • Female
  • Gastrointestinal Diseases / diagnosis
  • Gastrointestinal Diseases / epidemiology*
  • Gastrointestinal Diseases / physiopathology*
  • Health Surveys / methods
  • Humans
  • Joint Instability / diagnosis
  • Joint Instability / epidemiology
  • Joint Instability / physiopathology
  • Male
  • Middle Aged
  • Postural Orthostatic Tachycardia Syndrome / diagnosis
  • Postural Orthostatic Tachycardia Syndrome / epidemiology*
  • Postural Orthostatic Tachycardia Syndrome / physiopathology*
  • United Kingdom / epidemiology
  • Young Adult