Objective: To test the reproducibility of tests and criteria for generalized joint hypermobility (GJH) and benign joint hypermobility syndrome (BJHS).
Methods: A standardized protocol for clinical reproducibility studies was followed using a three-phase study (with a training, an overall agreement and a test phase). An overall agreement of at least 0.80 was required to proceed to the test phase. Phases 1, 2 and 3 used 14 patients (with varying degrees of hypermobility), 20 patients (50% cases) and 40 patients (50% cases), respectively. The inclusion criterion for cases was hypermobility (patients with Ehlers-Danlos Syndrome or BJHS) and for controls, non-hypermobility (patients with shoulder and/or back pain); patients were selected from patients' files (phases 1 and 2) or included consecutively from our outpatient clinic (phase 3).
Results: The overall agreement in phase 2 was 0.95 for GJH and 0.90 for BJHS. Reproducibility for diagnosing GJH and BJHS in phase 3 showed kappa values of 0.74 and 0.84, respectively. Kappa in the Beighton tests for diagnosing GJH (currently or historically) was generally above 0.80, except for the fifth fingers and elbows (> or = 0.60). In the Brighton tests for diagnosing BJHS, kappa was above 0.73, except for the skin signs (0.63). Lowest kappa was found in the Rotès-Quérol tests, where it was > or = 0.57, except for the right shoulder (0.31).
Conclusion: We found a good-to-excellent reproducibility of tests and criteria for GJH and BJHS. Future research on the validity of the tests and criteria for joint hypermobility is urgently needed.