Association between Ehlers-Danlos syndrome and complex regional pain syndrome: a large nationwide claims database study

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BACKGROUND: Ehlers-Danlos syndrome (EDS) is a genetic connective tissue disorder characterized by joint instability and recurrent injuries, which has been linked to chronic pain syndromes, including complex regional pain syndrome (CRPS). This study examines the co-occurrence of EDS and CRPS and evaluates demographic differences using a large national claims database, with findings interpreted as population-level signal detection based on diagnostic coding. METHODS: This retrospective cohort study used 2017-2022 Merative MarketScan data (IRB# 2017-0169), including commercially insured US patients. Individuals with and without EDS (ICD-10-CM: Q79.6x) were assessed for CRPS diagnoses (ICD-10-CM: G90.5x, G56.4x, G57.7x). Sensitivity analysis was conducted with all patients to investigate associations between CRPS and EDS. RESULTS: Of 54,235,476 patients, 26,053 (0.05%) had EDS, and 51,424 (0.09%) had CRPS. There were 273 patients with a CRPS diagnosis after an EDS diagnosis, 217 with a CRPS diagnosis before an EDS diagnosis, and 490 with co-occurrence of EDS and CRPS, regardless of the order of diagnosis. The proportion of recorded CRPS diagnoses was higher among patients with recorded EDS diagnoses (1.05%; 273/26,053) compared with patients without EDS (0.09%; 51,151/54,158,272), corresponding to an approximately 11-fold higher proportion of recorded CRPS diagnoses among patients with EDS (95% CI 9.89 to 12.51). Among patients with EDS, those with recorded CRPS diagnoses and those without recorded CRPS diagnoses were predominantly female (96.7% vs 81.3%), with a median age of 27 years. Among CRPS cases, 76.8% had type 1 disease, and lower-limb involvement was most frequent (49.0%). CONCLUSIONS: Patients with EDS had a substantially higher proportion of recorded CRPS diagnoses than patients without EDS in this national claims dataset. These findings support further investigation into the observed association between EDS and CRPS diagnostic coding.

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