title: Mechanisms underlying reflux symptoms and dysphagia in patients with joint hypermobility syndrome, with and without postural tachycardia syndrome.
abstract: BACKGROUND
  The joint hypermobility syndrome (JHS) is a common non-inflammatory connective tissue disorder which frequently co-exists with postural tachycardia syndrome (PoTS), a form of orthostatic intolerance. Gastrointestinal symptoms and dysmotility have been reported in PoTS. Dysphagia and reflux are common symptoms in JHS, yet no studies have examined the physiological mechanism for these, subdivided by PoTS status.
  
  
  METHODS
  Thirty patients (28 female, ages: 18-62) with JHS and symptoms of reflux (n=28) ± dysphagia (n=25), underwent high-resolution manometry and 24 hour pH-impedance monitoring after questionnaire-based symptom assessment. Esophageal physiology parameters were examined in JHS, subdivided by PoTS status.
  
  
  RESULTS
  Fifty-three percent of JHS patients with reflux symptoms had pathological acid reflux, 21% had reflux hypersensitivity, and 25% had functional heartburn. Acid exposure was more likely to be increased in the recumbent than upright position (64% vs 43%). The prevalence of hypotensive lower esophageal sphincter (33%) and hiatus hernia (33%) was low. Forty percent of patients with dysphagia had minor disorders of motility, 60% had functional dysphagia. Eighteen (60%) patients had coexistent PoTS-they had significantly higher dysphagia (21 vs 11.5, P=.04) and reflux scores (24.5 vs 16.5, P=.05), and double the prevalence of pathological acid reflux (64% vs 36%, P=.1) and esophageal dysmotility (50% vs 25%, P=.2) though this was not significant.
  
  
  CONCLUSIONS
  A large proportion of JHS patients with esophageal symptoms have true reflux-related symptoms or mild esophageal hypomotility, and this is more likely if they have PoTS.
authors.affiliation: Wingate Institute of Neurogastroenterology, Centre for Digestive Diseases, Blizard Institute of Cell and Molecular Science, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London, UK.
authors.forename: A
authors.initials: A
authors.lastname: Fikree
authors.affiliation: Wingate Institute of Neurogastroenterology, Centre for Digestive Diseases, Blizard Institute of Cell and Molecular Science, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London, UK.
authors.forename: Q
authors.initials: Q
authors.lastname: Aziz
authors.affiliation: Wingate Institute of Neurogastroenterology, Centre for Digestive Diseases, Blizard Institute of Cell and Molecular Science, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London, UK.
authors.forename: D
authors.initials: D
authors.lastname: Sifrim
mesh_terms: D000328:Adult; D003680:Deglutition Disorders / Q000150:complications / Q000503:physiopathology*; D050782:Esophageal pH Monitoring; D005260:Female; D005764:Gastroesophageal Reflux / Q000150:complications / Q000503:physiopathology*; D005769:Gastrointestinal Motility; D006801:Humans; D007593:Joint Instability / Q000150:complications / Q000503:physiopathology*; D008297:Male; D008365:Manometry; D054972:Postural Orthostatic Tachycardia Syndrome / Q000150:complications / Q000503:physiopathology*
keywords: Ehlers-Danlos syndrome; dysmotility; functional gastrointestinal disorder; high-resolution manometry; postural tachycardia syndrome