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Psychiatric disorders in Ehlers–Danlos syndrome are frequent, diverse and strongly associated with pain

  • Original Article - Observational Research
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Abstract

Ehlers–Danlos syndromes (EDS) are a heterogeneous group of hereditary connective tissue disorders characterized by joint hypermobility, widespread musculoskeletal pain and tissue fragility. Psychiatric disorders and psychosocial impairment are common, yet poorly characterized, findings in EDS patients. We investigated the frequency and types of psychiatric disorders and their relationship to systemic manifestations in a cohort of 106 classic and hypermobility type EDS patients. In this retrospective study, extensive medical chart review was performed for patients referred at two genetics clinics who were diagnosed with EDS. Statistical analysis was undertaken to determine the frequency of psychiatric disorders and association with systemic findings. Psychiatric disorders were found in 42.5 % of the EDS cohort, with 22.7 % of patients affected with 2 or more psychiatric diagnoses. Anxiety and depression were most commonly reported, with frequencies of 23.6 and 25.5 %, respectively. A variety of other psychiatric diagnoses were also identified. Abdominal pain [odds ratio (OR) 7.38], neuropathic pain (OR 4.07), migraines (OR 5.21), joint pain (OR 2.85) and fatigue (OR 5.55) were significantly associated with the presence of a psychiatric disorder. The presence of any pain symptom was significantly associated with having a psychiatric disorder (OR 9.68). Muscle pain (OR 2.79), abdominal pain (OR 5.78), neuropathic pain (OR 3.91), migraines (OR 2.63) and fatigue (OR 3.78) were significantly associated with having an anxiety or mood disorder. Joint hypermobility and the classic dermatological features of EDS showed no significant association with having a psychiatric disorder. Our findings demonstrate a high frequency of psychiatric disorders and an association with pain symptoms in EDS.

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Acknowledgments

The authors would like to thank all of the EDS patients included in this study. S.H. and V.M. were funded by the Mach-Gaensslen Foundation of Canada.

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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

    Samantha Aliza Hershenfeld, Syed Wasim, Vanda McNiven, Manasi Parikh, Hanna Faghfoury & Joyce So

  2. Clinical Genetics, Lakeridge Health, Oshawa, Canada

    Paula Majewski & Joyce So

  3. Department of Medicine, University of Toronto, Toronto, Canada

    Hanna Faghfoury

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

    Joyce So

  5. Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Canada

    Joyce So

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  1. Samantha Aliza Hershenfeld
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Correspondence to Joyce So.

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Hershenfeld, S.A., Wasim, S., McNiven, V. et al. Psychiatric disorders in Ehlers–Danlos syndrome are frequent, diverse and strongly associated with pain. Rheumatol Int 36, 341–348 (2016). https://doi.org/10.1007/s00296-015-3375-1

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