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Sci Rep. 2017 Jan 4;7:39636. doi: 10.1038/srep39636.

Ehlers-Danlos syndrome hypermobility type is associated with rheumatic diseases.

Author information

1
Department of Medicine, Geisel School of Medicine at Dartmouth, Lebanon, 03756, NH, USA.
2
Department of Biomedical Data Science, Geisel School of Medicine at Dartmouth, Lebanon, 03756, NH, USA.
3
Department of Community and Family Medicine, Geisel School of Medicine at Dartmouth, Lebanon, 03756, NH, USA.
4
The Dartmouth Institute for Health Policy and Clinical Practice, Lebanon, NH, USA.
5
Department of Pediatrics, Geisel School of Medicine at Dartmouth, Lebanon, 03756, NH, USA.
6
Department of Pathology, Geisel School of Medicine at Dartmouth, Lebanon, 03756, NH, USA.
7
Division of Genetics, Dartmouth-Hitchcock Medical Center, Lebanon, 03756, NH, USA.
8
Division of Rheumatology, Dartmouth-Hitchcock Medical Center, Lebanon, 03756, NH, USA.

Abstract

We retrospectively analyzed electronic medical records of patients with Ehlers-Danlos Syndrome hypermobility type (HEDS), including demographic information, workup, rheumatological diagnoses in order to determine its association with rheumatological conditions. HEDS Patients were stratified according to level of workup received (no additional work (physical exam only) = NWU, limited workup = LWU, comprehensive workup = CWU)). HEDS patients were predominantly female (21:4, F:M). The percentage of patients with at least one rheumatological condition was significantly correlated with level of workup (NWU, 9.2%; LWU, 33.3%, CWU, 67.1%; p-value < 0.0001). The HLA-B27 antigen was more prevalent (p-value < 2.2 × 10-8) in the CWU HEDS patients (23.9%) than in the general population of the United States (6.1%). HEDS with CWU were associated with more rheumatological conditions (i.e. psoriasis, ankylosing spondylitis, rheumatoid arthritis, fibromyalgia) than those with NWU or LWU. In conclusion, HEDS is associated with complicated rheumatological conditions, which are uncovered by comprehensive workup. These conditions require different clinical management strategies than HEDS, and left untreated could contribute to the pain or even physical disability (i.e. joint erosions) in HEDS patients. While the mechanisms underlying these associations are unknown, it is important that all HEDS patients receive adequate workup to ensure a complete clinical understanding for the best care strategy possible.

PMID:
28051109
PMCID:
PMC5209734
DOI:
10.1038/srep39636
[Indexed for MEDLINE]
Free PMC Article

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