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Cited by 14Temporomandibular Joint DisordersConnective tissue disorders researchFibromyalgia and Chronic Fatigue Syndrome Research

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

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Willich, Leon, Bohner, Lauren, Köppe, Jeanette, Jackowski, Jochen, Hanisch, Marcel, Oelerich, Ole·2023·Orphanet Journal of Rare Diseases
DOI 10.1186/s13023-023-02877-1PMC PMC10510186
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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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  • Three studies including all adult patients with symptomatic GJH or hEDS older than 18 years reported similar prevalences of anxiety as the symptomatic groups.

    “Three studies that overlapped in age-groups by including all adult patients with symptomatic GJH, hEDS older than 18 years reported similar prevalences of anxiety39,50,58 as the symptomatic groups.”

    From Role of Anxiety in Individuals with Generalized Joint Hypermobility: A Systematic Review (2025) · cites this paper as [58]

    The Results (Participants; Psychological stress factors, page unavailable) show an adult cohort including 230 people with hEDS and report that 53.9% of the hEDS participants reached the anxiety threshold. The paper also includes 29 people with classical EDS, and our copy does not compare its anxiety prevalence with the citing document’s “symptomatic groups.” (LLM, conf=0.84)

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Cited by

Sorted by citations

Authors
Willich, L., Bohner, L., Köppe, J., Jackowski, J., Hanisch, M., & Oelerich, O.
Affiliations
  • University Hospital Münster (DE)
  • University of Münster (DE)
  • Witten/Herdecke University (DE)
Corresponding author
  • Hanisch, Marcel
  • Oelerich, Ole
ORCID
Venue
Orphanet Journal of Rare Diseases
Publisher
Springer Science and Business Media LLC
Published
2023-09-19
Volume
18
Issue
1
Pages
294-294
ISSN
1750-1172 (ISSN-L)
DOI
10.1186/s13023-023-02877-1
PMC
PMC10510186
OpenAlex
W4386861701
Cited by
14
References
11
Citations by year
2026: 3 · 2025: 6 · 2024: 5
FWCI
4.09
Type
journal article
Language
en
Open access
Licence
Locations
  • Orphanet Journal of Rare Diseases (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Funders
Universitätsklinikum Münster
Topics
  • Health Sciences › Health Professions › Complementary and Manual Therapy › Temporomandibular Joint Disorders
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
  • Health Sciences › Medicine › Psychiatry and Mental health › Fibromyalgia and Chronic Fatigue Syndrome Research
Keywords
Ehlers-Danlos syndrome, hypermobile Ehlers-Danlos syndrome, classical Ehlers-Danlos syndrome, temporomandibular disorders, chronic pain, psychological distress, depression, anxiety, stress, masticatory muscle pain, connective tissue diseases, prevalence
MeSH terms
Psychological Distress, Ehlers-Danlos Syndrome, Humans, Prevalence, Chronic Pain

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