Ehlers-Danlos syndrome (EDS) is a group of genetic disorders that affect connective tissue, including the skin, joints, and blood vessel walls. Characteristics and symptoms vary depending on the specific type of EDS, but often include joint hypermobility, skin hyperextensibility, tissue fragility, and a tendency toward other systemic conditions. Hypermobile Ehlers-Danlos syndrome (hEDS) is the most common type of EDS, characterized further by joint instability and chronic pain.1-3
Conversely, hypermobility spectrum disorders (HSDs) are characterized by joint hypermobility and other associated symptoms that do not meet the criteria for hEDS.1
Both conditions, hEDS and HSD, contribute to a higher prevalence of temporomandibular joint disorders (TMDs), possibly due to general joint hypermobility. Of those with EDS, the estimated prevalence ranges from 27%–100%, with hEDS being the most commonly reported subtype associated with TMD. The relationship between TMD and joint hypermobility involves biomechanical, neuromuscular, and structural factors. Women are more likely to be affected by TMD, even those without EDS. Increased incidence of TMD in women is suspected to be associated with hormonal factors, but also, joint hypermobility is more common in women than in men.1
TMD often affects the quality of life, including impaired eating and speaking, pain that leads to mood disturbances, lower sleep quality, and impaired oral-health-related quality of life (OHRQoL). Patient-reported OHRQoL is often used to assess a patient’s level of impairments and is frequently used in dental research.1
A widely used measure for assessing OHRQoL is the Oral Health Impact Profile (OHIP). Previous studies using the OHIP have evaluated TMD in mixed EDS groups that included men and women, despite the knowledge that women experience more severe complications than men. Only one study has assessed the impact of EDS on women’s quality of life. However, this included mixed EDS subgroups and did not consider TMD as a factor.1
Previous studies have also reported that the EDS population has poorer OHRQoL than the general population. However, the metrics used in previous studies have not evaluated specific demographics and specific subsets of EDS. This leaves a gap in knowledge regarding women and individuals with hEDS or HSD who are more often affected.1
A study aimed to address this gap by evaluating “OHRQoL and associated risk factors in women with self-reported TMD symptoms and confirmed hEDS or HSD.”1
The Study
The study design was cross-sectional, using data from The Swedish National EDS Association. The data was used to identify individuals with confirmed or suspected EDS or HSD from January through March of 2022.1
A questionnaire was emailed to individuals identified in the data collection process. Nine hundred fifty individuals were sent the digital questionnaire, and 279 initially responded. The questionnaire consisted of 63 questions on various metrics, including sociodemographic factors, general and oral health factors, EDS type or HSD, comorbid symptoms, psychological factors, symptoms attributed to TMD, and a 14-item short version of the OHIP (OHIP-14).1
Self-reported TMD symptoms were categorized into two sections: pain and dysfunction, assessed through five yes-or-no questions. TMD pain was considered present when at least one positive response was recorded to the two pain questions. TMD symptoms were considered present when either pain or dysfunction symptoms, such as TMJ clicking, crepitation, and luxation, were reported.1
The OHRQoL was assessed using the OHIP-14, a 5-point rating scale (0 = never, 1 = hardly ever, 2 = occasionally, 3 = fairly often, and 4 = very often) that covered a recall period of the past 30 days. The results were distributed into two groups: (1) never, hardly ever, and occasionally, and (2) often and very often.1
Additionally, four dimensions of OHRQoL (oral function, orofacial pain, orofacial appearance, and psychosocial impact) were calculated using related domains, including physical disability, physical pain, psychological discomfort, and factors related to overall life satisfaction and social participation.1
Each category’s score ranged from 0–8, with a total score of up to 56 for all categories combined. The higher the score, the higher the OHRQoL impairment.1
Age was grouped into two categories: adults less than 50 years and adults 50 years or older. Questions regarding general health could be answered with the following responses: very good, good, fair, poor, or very poor. Individuals who responded with fair, poor, and very poor were determined to have poor general health, while those who responded with very good and good were determined to have good general health.1
Self-reported oral health was assessed similarly to self-reported general health. Individuals reported their number of teeth, which was then divided into two categories: 0–24 teeth and 25–32 teeth. Bruxism was determined by asking two yes or no questions. Yes, responses were considered a positive indication of bruxism.1
- Do you usually grind your teeth?
- Do you often clench your teeth?
A single question was used to determine bothersome head, neck, arm, upper back, lower back, stomach, feet, elbow, wrist, hip, knee, and ankle pain. Participants could choose the pain level and the most bothersome area of pain. The location of pain was then categorized into limbs or joints.1
Four questions were asked to determine distress as the construct of anxiety and depression. Responses indicating none or mild were designated to one category, and moderate and severe were designated to a separate category.1
Of the 279 individuals who initially responded, a sample of 137 had a confirmed diagnosis of hEDS or HSD. Three individuals were excluded because they did not report TMD symptoms, and one was excluded due to missing data in the OHIP-14 section of the questionnaire. This left a total sample size of 133 women aged 24–78 years.1
The Results
Of the sample with confirmed hEDS and HSD, the most common symptoms reported included feeling tense, painful aching in the mouth, discomfort when eating, and difficulty relaxing. They also reported lower satisfaction with life. Participants with hEDS had a 2.5 times increased risk of dysfunctional pain associated with TMD.1
Almost 94% of the participants reported TMD pain symptoms, about 90% reported TMJ clicking, and about 56% reported crepitation. Most participants reported good oral health, with more than 24 teeth, but most also reported bruxism. In contrast, most participants reported poor general health, with about 47% reporting bothersome pain in the limb region (head, neck, arm, upper back, lower back, stomach, or feet). Almost 62% reported moderate or severe distress rates. Of the 133 participants, only 10 rated their health as good or very good. Poor general health has been associated with impaired OHRQoL.1
Oral function had the lowest impact on OHRQoL, while orofacial pain had the highest impact on OHRQoL. Additionally, pain reported in the limbs or joints had less effect on OHRQoL than bruxism and poor general health. The participants of this study reported experiencing many impairments that impacted their quality of life. This finding is in line with previous studies of patients with EDS.1
Notably, patients with EDS had a wide range of systemic symptoms and psychological issues. Of those, the most reported were malocclusion, TMD, anxiety, and feelings of insecurity regarding teeth, dentures, or mouth, which may be factors related to physical orofacial pain and oral dysfunction.1
Almost 93% of the women in this study reported bruxism. However, the authors note that the wording of the questions associated with bruxism could have influenced the response, leading to an overestimation of the prevalence of bruxism.1
This study had multiple limitations, including a low participation rate, a risk of selection bias, a risk of recall bias, and a lack of a control group. The strengths of this study included the holistic approach, good reliability, and validity.1
Conclusion
The OHRQoL of women with hEDS and HSD with TMD is multifactorial. It is influenced by bruxism, poor general health, and comorbid symptoms. These factors should be considered when treating and managing TMD in patients with hEDS and HSD.1
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References
- Yekkalam, N., Sipilä, K., Novo, M., et al. Oral Health-Related Quality of Life among Women with Temporomandibular Disorders and Hypermobile Ehlers-Danlos Syndrome or Hypermobility Spectrum Disorder. J Am Dent Assoc. 2024; 155(11): 945-953. https://jada.ada.org/article/S0002-8177(24)00496-3/fulltext
- What Is EDS? (n.d.). The Ehlers-Danlos Society. https://www.ehlers-danlos.com/what-is-eds/
- Hypermobile Ehlers-Danlos Syndrome (hEDS). (n.d.). The Ehlers-Danlos Society. https://www.ehlers-danlos.com/heds/
