title: Psychiatric disorders in Ehlers-Danlos syndrome are frequent, diverse and strongly associated with pain.
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.
authors.affiliation: 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.
authors.forename: Samantha Aliza
authors.initials: SA
authors.lastname: Hershenfeld
authors.affiliation: 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.
authors.forename: Syed
authors.initials: S
authors.lastname: Wasim
authors.affiliation: 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.
authors.forename: Vanda
authors.initials: V
authors.lastname: McNiven
authors.affiliation: 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.
authors.forename: Manasi
authors.initials: M
authors.lastname: Parikh
authors.affiliation: Clinical Genetics, Lakeridge Health, Oshawa, Canada.
authors.forename: Paula
authors.initials: P
authors.lastname: Majewski
authors.affiliation: 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.|Department of Medicine, University of Toronto, Toronto, Canada.
authors.forename: Hanna
authors.initials: H
authors.lastname: Faghfoury
authors.affiliation: 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. joyce.so@utoronto.ca.|Clinical Genetics, Lakeridge Health, Oshawa, Canada. joyce.so@utoronto.ca.|Centre for Addiction and Mental Health, Toronto, Canada. joyce.so@utoronto.ca.|Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Canada. joyce.so@utoronto.ca.
authors.forename: Joyce
authors.initials: J
authors.lastname: So
mesh_terms: D015746:Abdominal Pain / Q000175:diagnosis / Q000453:epidemiology* / Q000523:psychology; D000328:Adult; D018771:Arthralgia / Q000175:diagnosis / Q000453:epidemiology* / Q000523:psychology; D015897:Comorbidity; D004535:Ehlers-Danlos Syndrome / Q000175:diagnosis / Q000453:epidemiology* / Q000523:psychology; D005260:Female; D006801:Humans; D008297:Male; D001523:Mental Disorders / Q000175:diagnosis / Q000453:epidemiology* / Q000523:psychology; D008875:Middle Aged; D008881:Migraine Disorders / Q000175:diagnosis / Q000453:epidemiology* / Q000523:psychology; D009437:Neuralgia / Q000175:diagnosis / Q000453:epidemiology* / Q000523:psychology; D016017:Odds Ratio; D009864:Ontario / Q000453:epidemiology; D010147:Pain Measurement; D011569:Psychiatric Status Rating Scales; D012189:Retrospective Studies; D012307:Risk Factors; D055815:Young Adult
keywords: Anxiety; Connective tissue disorders; Depression; Ehlers–Danlos syndrome; Pain; Psychiatric disorders