Background Ehlers Danlos Syndrome, hypermobility type(EDS-HT) is characterized by overall connective tissue laxity and associated with chronic pain, fatigue and subsequent disability. Literature reports that EDS-HT also has profound effects on the level of psychological well being. The incidence of anxiety related disorders is more prevalent in individuals with EDS-HT in comparison to other populations. In addition, maladaptive coping have also been reported. Still in EDS-HT no evidence is available regarding the consequences of psychological comorbidity on activity impairments and it remains unclear how this is influenced by the way patients cope with their disability.

Objectives To establish the association between pain and activity impairments controlled for psychological comorbidity and coping in patients with EDS-HT.

Methods 25 patients with EDS-HT according to the Villefranche criteria were included and compared to 25 age and gender matched controls. Activity impairments, on capacity qualifiers, were quantified according to the Health Assessment Questionaire (HAQ) served as dependent variables. Experienced pain measured by the Visual Analog Scale (VAS) was used as independent variable. Coping measured by the Pain Catastrophizing Scale (PCS) and the Pain Vigilance and Awareness Questionnaire (PVAQ) were inserted as independent factors into the model. Fatigue (checklist individual strength: CIS20), anxiety (Hospital Anxiety and Depression Scale Anxiety: HADSA) and subject demographics (age, BMI) were inserted in the model as potential confounders. Models were calculated using GEE analysis.

Results Univariate analysis performed with pearson correlations, revealed significant associations between pain and HAQ(r=.830, p=<.0001), PCS (r=.468, p=.001) and PVAQ (r=.454, p=.001) and HADSA (r=.420, p=.003). Multivariate analysis showed significant associations between HAQ and VAS(B(SE):.367(0.1), p= 0.001) when controling for age, BMI and fatigue. This effect was modified by pain coping strategies in terms of PCS(B(SE):.185(0.1), p=<0.0001) and PVAQ(B(SE); -.113(0.1), p=<.0001). When regarding the association between HAQ and VAS, controling for psychologic comorbidity(HADSA), again activity impairment was associated with pain(B(SE):.306(0.1), p=<.0001). Psychologic comorbidity was a significant independent contributor to activity impairment(B(SE): .031(0.1), p=<.0001).

Conclusions In EDS-HT presence of pain, controlled both for coping and psychological comorbidities, is significant associated with activity impairments. EDS-HT patients who experience more pain are less independent in their ADL activities. Furthermore, this demonstrates that coping strategies play an important role in the psychological well being of a patient with EDS-HT, but also have profound consequences for the amount of disability. These insights might be usefull for the development of new treatment modalities, especially for chronically disabled individuals.

References: Baeza-Velasco C, Gély-Nargeot MC, Bulbena et al. Joint hypermobility syndrome: problems that require psychological intervention. Rheumatol Int. 2011;31(9):1131-6.

Voermans NC, Knoop H, Bleijenberg G, et al. Pain in ehlers-danlos syndrome is common, severe, and associated with functional impairment. J Pain Symptom Manage. 2010;40(3):370-8.

Disclosure of Interest None Declared