Pain in ehlers-danlos syndrome is common, severe, and associated with functional impairment.
BACKGROUND
The Ehlers-Danlos Syndrome (EDS) is a clinically and genetically heterogeneous group of heritable connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Musculoskeletal pain is mentioned in the diagnostic criteria and described as early in onset, chronic, and debilitating. However, systematic research on pain in EDS is scarce.


OBJECTIVE
We investigated prevalence and impact of pain and associated features in a large group of EDS patients.


METHODS
We performed a study among members of the Dutch EDS patient organization (n=273) and included the McGill Pain Questionnaire to investigate various aspects of pain, the Sickness Impact Profile to study functional impairment, the Symptom Checklist subscale sleep to evaluate sleep disturbances, and the Checklist Individual Strength subscale fatigue to determine fatigue severity.


RESULTS
The results of this study show that 1) chronic pain in EDS is highly prevalent and associated with regular use of analgesics; 2) pain is more prevalent and more severe in the hypermobility type than in the classic type; 3) pain severity is correlated with hypermobility, dislocations, and previous surgery; 4) pain is correlated with low nocturnal sleep quality; and 5) pain contributes to functional impairment in daily life, independent of the level of fatigue.


CONCLUSIONS
From this large cohort of EDS patients, we conclude that pain is common and severe in EDS. Pain is related to hypermobility, dislocations, and previous surgery and associated with moderate to severe impairment in daily functioning. Therefore, treatment of pain should be a prominent aspect of symptomatic management of EDS.
Voermans
Nicol C
NC
Department of Neurology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. n.voermans@neuro.umcn.nl
Knoop
Hans
H
Bleijenberg
Gijs
G
van Engelen
Baziel G
BG
D000203:Activities of Daily Living; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000700:Analgesics / Q000627:therapeutic use; D003430:Cross-Sectional Studies; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000523:psychology*; D005221:Fatigue / Q000209:etiology / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010146:Pain / Q000453:epidemiology / Q000209:etiology* / Q000523:psychology*; D010147:Pain Measurement; D012893:Sleep Wake Disorders / Q000209:etiology; D055815:Young Adult
D000700:Analgesics