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.