In this retrospective study, we investigate the frequency and types of psychiatric disorders and their relationship to systemic manifestations in a cohort of 391 Ehlers–Danlos syndromes (EDS) and hypermobility spectrum disorder (HSD) patients based on the current 2017 International Classification of EDS diagnostic criteria. A detailed, systematic retrospective chart review was undertaken for patients assessed for HSD or EDS at two Canadian health centres. Patients were diagnosed according to the Villefranche criteria and reclassified for this study according to the 2017 International Classification of EDS. Data validation and statistical analyses were conducted. Psychiatric disorders were very common, with 49.4% of the total cohort affected; 28.9% reported multiple psychiatric diagnoses. Mood (34.5%) and somatoform (28.6%) disorders were most common. Interestingly, attention-deficit/hyperactivity disorder (ADHD) was significantly enriched in the HSD, but not EDS cohort (p = 0.0002, 95% CI 3.48–9.00) compared to the general population. There were no differences in the systemic associations with having psychiatric manifestations in the HSD compared to the EDS subsets. Muscle/body pain (OR 1.99) and gastrointestinal dysfunction (OR 2.07) were significantly associated with having mood disorders, and gastrointestinal dysfunction (OR 2.61) and nerve-related pain (OR 3.27) were associated with having somatoform disorders across the cohort. The common systemic associations with the presence of psychiatric manifestations in both HSD and EDS reaffirm that the conditions should be treated as a spectrum rather than as wholly separate entities, particularly with respect to psychiatric management. EDS and HSD patients share common psychiatric presentations, though ADHD is more common with HSD.
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We acknowledge Dorsa Sohaei for assisting with data collection.
The authors declare that this work was not supported by any grant or funding.
Conflict of interest
All authors declare no conflicts of interest.
Ethics approval for this study was obtained from University Health Network (Research Ethics Board (REB) #: 16-5634; date of approval: July 22, 2016), Mount Sinai Hospital (REB #: 16-0180-C; date of approval: August 8, 2016) and Queen’s University (REB #: PAED-406-16; date of approval: September 27, 2016).
Waiver of informed consent was obtained from the University Health Network, Mount Sinai Hospital and Queen’s University REBs.
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Comparing the frequency of study patients (in percentage) classified according to the Villefranche (old) classification and the 2017 International (new) Classification. Analyses in the current study were undertaken according to the current classification. *hcEDS was not used when classifying patients according to the new criteria. **Classic-like Ehlers–Danlos Syndrome (clEDS) is a new diagnosis in the 2017 International Classification. Abbreviations: HSD, hypermobility spectrum disorders. hEDS, hypermobile EDS. hcEDS, overlapping hypermobility and classic EDS. cEDS, classic EDS. clEDS, classical-like EDS, a, arthrochalasia EDS. v, vascular EDS. k, kyphoscoliotic EDS. (TIFF 4546 KB)
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Wasim, S., Suddaby, J.S., Parikh, M. et al. Pain and gastrointestinal dysfunction are significant associations with psychiatric disorders in patients with Ehlers–Danlos syndrome and hypermobility spectrum disorders: a retrospective study. Rheumatol Int 39, 1241–1248 (2019). https://doi.org/10.1007/s00296-019-04293-w
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