Prevalence and quality of temporomandibular disorders, chronic pain and psychological distress in patients with classical and hypermobile Ehlers-Danlos syndrome: an exploratory study

Abstract Background The Ehlers-Danlos syndromes are a group of clinically and genetically heterogeneous hereditary diseases affecting the connective tissue. They are characterized by hypermobility of the joints, hyperextensible skin and friable tissue. According to current classification, 13 subtype...

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Main Authors: Leon Willich, Lauren Bohner, Jeanette Köppe, Jochen Jackowski, Marcel Hanisch, Ole Oelerich
Format: Article
Language:English
Published: BMC 2023-09-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Online Access:https://doi.org/10.1186/s13023-023-02877-1
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author Leon Willich
Lauren Bohner
Jeanette Köppe
Jochen Jackowski
Marcel Hanisch
Ole Oelerich
author_facet Leon Willich
Lauren Bohner
Jeanette Köppe
Jochen Jackowski
Marcel Hanisch
Ole Oelerich
author_sort Leon Willich
collection DOAJ
description Abstract Background The Ehlers-Danlos syndromes are a group of clinically and genetically heterogeneous hereditary diseases affecting the connective tissue. They are characterized by hypermobility of the joints, hyperextensible skin and friable tissue. According to current classification, 13 subtypes can be distinguished, of which the hypermobile and the classical subtype are the most prevalent. This study aimed to evaluate patients with classical (cEDS) and hypermobile (hEDS) Ehlers-Danlos syndrome regarding temporomandibular disorder (TMD), chronic pain, and psychological distress. Methods Support groups from Germany, Austria, and Switzerland were contacted, and social media were used to recruit participants. Free text questions, the German version of the Depression Anxiety and Stress Scale (DASS), and the German version of the Graded Chronic Pain Status (GCPS) were used. Results 259 participants were included (230 hEDS/29 cEDS). At least 49.2% of the participants had painful or restricted jaw movements, and at least 84.9% had pain in the masticatory muscles, with 46.3% already having a diagnosed TMD. Multivariate analysis showed a significant correlation between TMJ involvement and chronic pain with a 2.5-fold higher risk of chronic pain with a diagnosed TMD. 22.8% of participants had a critical score for depression, 53.3% had a critical score for anxiety, and 34.0% had a critical score for stress. Conclusion There is a high prevalence of TMD problems and chronic pain in patients with cEDS and hEDS. The lack of knowledge about these problems can create psychological distress. More research is needed to provide adequate treatment for patients with EDS.
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spelling doaj.art-4442c6f5abb64dfb8ddff410f796f0392023-11-20T10:53:55ZengBMCOrphanet Journal of Rare Diseases1750-11722023-09-0118111110.1186/s13023-023-02877-1Prevalence and quality of temporomandibular disorders, chronic pain and psychological distress in patients with classical and hypermobile Ehlers-Danlos syndrome: an exploratory studyLeon Willich0Lauren Bohner1Jeanette Köppe2Jochen Jackowski3Marcel Hanisch4Ole Oelerich5Department of Prosthodontics, University Hospital MünsterDepartment of Oral and Maxillofacial Surgery, University Hospital MünsterInstitute of Biostatistics and Clinical Research, University of MünsterDepartment of Oral Surgery and Policlinical Ambulance, Faculty of Health, Witten/Herdecke UniversityDepartment of Oral and Maxillofacial Surgery, University Hospital MünsterDepartment of Prosthodontics, University Hospital MünsterAbstract Background The Ehlers-Danlos syndromes are a group of clinically and genetically heterogeneous hereditary diseases affecting the connective tissue. They are characterized by hypermobility of the joints, hyperextensible skin and friable tissue. According to current classification, 13 subtypes can be distinguished, of which the hypermobile and the classical subtype are the most prevalent. This study aimed to evaluate patients with classical (cEDS) and hypermobile (hEDS) Ehlers-Danlos syndrome regarding temporomandibular disorder (TMD), chronic pain, and psychological distress. Methods Support groups from Germany, Austria, and Switzerland were contacted, and social media were used to recruit participants. Free text questions, the German version of the Depression Anxiety and Stress Scale (DASS), and the German version of the Graded Chronic Pain Status (GCPS) were used. Results 259 participants were included (230 hEDS/29 cEDS). At least 49.2% of the participants had painful or restricted jaw movements, and at least 84.9% had pain in the masticatory muscles, with 46.3% already having a diagnosed TMD. Multivariate analysis showed a significant correlation between TMJ involvement and chronic pain with a 2.5-fold higher risk of chronic pain with a diagnosed TMD. 22.8% of participants had a critical score for depression, 53.3% had a critical score for anxiety, and 34.0% had a critical score for stress. Conclusion There is a high prevalence of TMD problems and chronic pain in patients with cEDS and hEDS. The lack of knowledge about these problems can create psychological distress. More research is needed to provide adequate treatment for patients with EDS.https://doi.org/10.1186/s13023-023-02877-1Ehlers-Danlos syndromeRare diseasesTemporomandibular disorderOral healthPsychological impairment
spellingShingle Leon Willich
Lauren Bohner
Jeanette Köppe
Jochen Jackowski
Marcel Hanisch
Ole Oelerich
Prevalence and quality of temporomandibular disorders, chronic pain and psychological distress in patients with classical and hypermobile Ehlers-Danlos syndrome: an exploratory study
Orphanet Journal of Rare Diseases
Ehlers-Danlos syndrome
Rare diseases
Temporomandibular disorder
Oral health
Psychological impairment
title Prevalence and quality of temporomandibular disorders, chronic pain and psychological distress in patients with classical and hypermobile Ehlers-Danlos syndrome: an exploratory study
title_full Prevalence and quality of temporomandibular disorders, chronic pain and psychological distress in patients with classical and hypermobile Ehlers-Danlos syndrome: an exploratory study
title_fullStr Prevalence and quality of temporomandibular disorders, chronic pain and psychological distress in patients with classical and hypermobile Ehlers-Danlos syndrome: an exploratory study
title_full_unstemmed Prevalence and quality of temporomandibular disorders, chronic pain and psychological distress in patients with classical and hypermobile Ehlers-Danlos syndrome: an exploratory study
title_short Prevalence and quality of temporomandibular disorders, chronic pain and psychological distress in patients with classical and hypermobile Ehlers-Danlos syndrome: an exploratory study
title_sort prevalence and quality of temporomandibular disorders chronic pain and psychological distress in patients with classical and hypermobile ehlers danlos syndrome an exploratory study
topic Ehlers-Danlos syndrome
Rare diseases
Temporomandibular disorder
Oral health
Psychological impairment
url https://doi.org/10.1186/s13023-023-02877-1
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