Shoulder function, pain and health related quality of life in adults with joint hypermobility syndrome/Ehlers-Danlos syndrome-hypermobility type.

Purpose To investigate shoulder function, pain and Health-Related Quality of life (HRQoL) among adults with joint hypermobility syndrome/Ehlers-Danlos syndrome-hypermobility type (JHS/EDS-HT), compared with the general population (controls). Method In a cross-sectional study using postal survey, 110 patients diagnosed with JHS/EDS-HT and 140 gender- and age-matched healthy controls from Statistics Norway participated. Shoulder function, pain and HRQol were registered by Western Ontario Shoulder Instability Index (WOSI), Numerical Rating Scale (NRS), pain drawings, 36-item Short Form (SF-36). Results Eighty-one individuals responded, with response rate 34% (JHS/EDS-HT: 53%, controls: 21%). JHS/EDS-HT had lower shoulder function (WOSI total: 49.9 versus 83.3; p < 0.001), lower HRQol on SF-36 Physical Component Scale (PCS: 28.1 versus 49.9; p < 0.001), and higher pain intensity (NRS: 6.4 versus 2.7; p < 0.001) than controls. Neck and shoulder joints were rated as primary painful areas in both groups, with significantly higher frequency in JHS/EDS-HT (neck: 90% versus 27%; shoulder: 80% versus 37%). Further, JHS/EDS-HT most often reported generalized pain (96%). Conclusions Adults with JHS/EDS-HT have impaired shoulder function, increased pain intensity, as well as reduced physical HRQoL compared with controls. Although neck and shoulder were most frequently rated as painful, significantly more JHS/EDS-HT also reported generalized pain compared to controls. Implications for Rehabilitation Adults with JHS/EDS-HT have impaired shoulder function, and most often painful areas in the neck and shoulder joints, which need to be targeted in the treatment strategy. Compared with the general population adults with JHS/EDS-HT have reduced physical HRQoL, supporting a physical approach for this group. Adults with JHS/EDS-HT may present with both specific painful joints and generalized pain.