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Pain and gastrointestinal dysfunction are significant associations with psychiatric disorders in patients with Ehlers–Danlos syndrome and hypermobility spectrum disorders: a retrospective study

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Abstract

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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Acknowledgements

We acknowledge Dorsa Sohaei for assisting with data collection.

Funding

The authors declare that this work was not supported by any grant or funding.

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Author notes

  1. S. Wasim and J. S. Suddaby contributed equally to this paper.

Authors and Affiliations

  1. Fred A. Litwin Family Centre in Genetic Medicine, University Health Network and Mount Sinai Hospital, 60 Murray Street, Box 34, 3rd Floor, Room 400, Toronto, ON, M5T 3L9, Canada

    S. Wasim, J. S. Suddaby, M. Parikh, S. Leylachian, B. Ho & J. So

  2. Department of Pediatrics, Division of Medical Genetics, Queen’s University, Kingston, Canada

    B. Ho & A. Guerin

  3. Centre for Addiction and Mental Health, Toronto, Canada

    J. So

  4. Departments of Medicine, Psychiatry, and Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Canada

    J. So

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Contributions

SW contributed to study design, data analysis and interpretation, and revision of the work. JSS contributed to data interpretation, and drafting and revision of the work. MP contributed to data acquisition, and drafting and revision of the work. SL, BH and AG contributed to data acquisition and revision of the work. JS contributed to study conception and design, data acquisition and interpretation, and drafting and revision of the work. All authors gave final approval of the version to be published and agree to be accountable for all aspects of the work.

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Correspondence to J. So.

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All authors declare no conflicts of interest.

Ethical approval

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).

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Waiver of informed consent was obtained from the University Health Network, Mount Sinai Hospital and Queen’s University REBs.

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296_2019_4293_MOESM1_ESM.tiff

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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