{
  "abstract": "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.",
  "authors": [
    {
      "affiliations": [
        "Icahn School of Medicine at Mount Sinai, New York, New York, USA",
        "Pain Prevention Research Center, Department of Anesthesiology, Critical Care, & Pain Management, Hospital for Special Surgery, New York, New York, USA"
      ],
      "name": "Renee Ren"
    },
    {
      "affiliations": [
        "Pain Prevention Research Center, Department of Anesthesiology, Critical Care, & Pain Management, Hospital for Special Surgery, New York, New York, USA"
      ],
      "name": "Tina Chen"
    },
    {
      "affiliations": [
        "Pain Prevention Research Center, Department of Anesthesiology, Critical Care, & Pain Management, Hospital for Special Surgery, New York, New York, USA"
      ],
      "name": "Alex Illescas"
    },
    {
      "affiliations": [
        "Pain Prevention Research Center, Department of Anesthesiology, Critical Care, & Pain Management, Hospital for Special Surgery, New York, New York, USA",
        "Department of Anesthesiology, Mount Sinai Health System, New York, New York, USA"
      ],
      "name": "Claire Yin"
    },
    {
      "affiliations": [
        "Pain Prevention Research Center, Department of Anesthesiology, Critical Care, & Pain Management, Hospital for Special Surgery, New York, New York, USA"
      ],
      "name": "William Chan"
    },
    {
      "affiliations": [
        "Anesthesiology, Critical Care and Pain Management, Hospital for Special Surgery, New York, New York, USA"
      ],
      "name": "Burcu Candan"
    },
    {
      "affiliations": [
        "Orthopaedics / Population Health Science & Policy, Hospital for Special Surgery, New York, New York, USA"
      ],
      "name": "Jashvant Poeran"
    },
    {
      "affiliations": [
        "Division of Pain Medicine, Department of Anesthesiology, Critical Care and Pain Management, Hospital for Special Surgery, New York, New York, USA",
        "Department of Anesthesiology, Weill Cornell Medicine, New York, New York, USA"
      ],
      "name": "Anuj Malhotra"
    },
    {
      "affiliations": [
        "Department of Anesthesiology, Weill Cornell Medicine, New York, New York, USA",
        "Anesthesiology, Critical Care & Pain Management, Hospital for Special Surgery, New York, New York, USA"
      ],
      "name": "Stavros G Memtsoudis"
    },
    {
      "affiliations": [
        "Pain Prevention Research Center, Department of Anesthesiology, Critical Care, & Pain Management, Hospital for Special Surgery, New York, New York, USA"
      ],
      "name": "Alexandra Sideris"
    },
    {
      "affiliations": [
        "Division of Pain Medicine, Department of Anesthesiology, Critical Care and Pain Management, Hospital for Special Surgery, New York, New York, USA",
        "Department of Anesthesiology, Weill Cornell Medicine, New York, New York, USA"
      ],
      "name": "Semih Gungor"
    }
  ],
  "title": "Association between Ehlers-Danlos syndrome and complex regional pain syndrome: a large nationwide claims database study",
  "uid": "7b7c8aef-474e-52cc-9303-89770c3e1a3d"
}